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Oncostomatology: statistics, organization of medical service, centre system of health. Classification of tumors of maxillofacial area. High qualities tumors.

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Presentation on theme: "Oncostomatology: statistics, organization of medical service, centre system of health. Classification of tumors of maxillofacial area. High qualities tumors."— Presentation transcript:

1 Oncostomatology: statistics, organization of medical service, centre system of health. Classification of tumors of maxillofacial area. High qualities tumors and tumor-like formations of soft tissue of maxillofacial area (papilloma, fibroma, lіpoma, hemangіoma, ateroma, neyrofіbromatozis, cysts of soft tissue): etiology, pathogenesis, histological structure, clinic, differential diagnostics, treatment and prophylaxis of complications.

2 Traumatic fibroma

3 Traumatic (Irritation) Fibroma: Clinical Features Traumatic fibromas appear as firm, asymptomatic nodules covered by epithelium unless secondarily traumatized. Traumatic fibromas appear as firm, asymptomatic nodules covered by epithelium unless secondarily traumatized. They usually occur along the line of occlusion in the lower lip or buccal mucosa; they may, however, be found anywhere in the oral cavity. They usually occur along the line of occlusion in the lower lip or buccal mucosa; they may, however, be found anywhere in the oral cavity. These lesions, which are reactive hyperplasias rather than true neoplasms as suggested by the term fibroma, are very common oral lesions. These lesions, which are reactive hyperplasias rather than true neoplasms as suggested by the term fibroma, are very common oral lesions.

4 Traumatic (Irritation) Fibroma: Cause It is a reactive lesion to trauma or chronic irritation. It is a reactive lesion to trauma or chronic irritation. Although rare according to research studies, clinically similar lesions may be true neoplasms (fibromas). Although rare according to research studies, clinically similar lesions may be true neoplasms (fibromas).

5 Traumatic (Irritation) Fibroma: Treatment Surgical excision is the treatment of choice. Surgical excision is the treatment of choice.

6 Traumatic (Irritation) Fibroma: Significance This lesion is a reactive one which has limited growth potential. This lesion is a reactive one which has limited growth potential. No malignant transformation has been reported. No malignant transformation has been reported.

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12 Epulis Fissuratum Denture Injury Tumor Inflammatory Fibrous Hyperplasia Denture Epulis

13 Epulis Fissuratum: Clinical Features Tumor-like hyperplasia of fibrous connective tissue associated with denture flange. Tumor-like hyperplasia of fibrous connective tissue associated with denture flange. Presents as fold (s), usually two, with flange fitting in between. Presents as fold (s), usually two, with flange fitting in between. The lesion is usually firm and fibrous but may show erythema and ulceration. The lesion is usually firm and fibrous but may show erythema and ulceration. Most common on facial aspect in the anterior of either jaw. Most common on facial aspect in the anterior of either jaw. It is more common in middle-aged and older females It is more common in middle-aged and older females (two-thirds to three-fourths of the cases are in females). (two-thirds to three-fourths of the cases are in females).

14 Epulis Fissuratum: Cause Chronic irritation or trauma from denture flange making the lesion a reactive hyperplasia. Chronic irritation or trauma from denture flange making the lesion a reactive hyperplasia. The denture is typically ill-fitting. The denture is typically ill-fitting.

15 Epulis Fissuratum: Treatment Treatment consists of surgical removal of excess tissue with microscopic examination and…. Treatment consists of surgical removal of excess tissue with microscopic examination and…. The poorly fitting denture should be remade or relined. The poorly fitting denture should be remade or relined.

16 Epulis Fissuratum: Significance Lesion will recur (or remain) if ill-fitting denture is not remade or relined. Lesion will recur (or remain) if ill-fitting denture is not remade or relined.

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25 Papillary Hyperplasia Inflammatory Papillary Hyperplasia Palatal Papillomatosis Denture Papillomatosis

26 (Inflammatory) Papillary Hyperplasia: Clinical Features Papillary hyperplasia is a reactive tissue growth that usually, but not always, develops beneath a denture. Papillary hyperplasia is a reactive tissue growth that usually, but not always, develops beneath a denture. It typically appears as a painless, papillomatous, “cobblestone” lesion of hard palate although occasionally it occurs on the edentulous mandibular ridge or in association with epulis fissuratum. It typically appears as a painless, papillomatous, “cobblestone” lesion of hard palate although occasionally it occurs on the edentulous mandibular ridge or in association with epulis fissuratum. The lesion is usually asymptomatic and red because of inflammation. The lesion is usually asymptomatic and red because of inflammation. It is a common lesion in denture wearers. It is a common lesion in denture wearers.

27 Papillary Hyperplasia: Cause Soft tissue reaction to ill-fitting denture and probable fungal overgrowth. Soft tissue reaction to ill-fitting denture and probable fungal overgrowth. Patients generally have poor oral/denture hygiene. Patients generally have poor oral/denture hygiene. One study indicated approximately 20 % of the patients wore their dentures 24 hours per day. One study indicated approximately 20 % of the patients wore their dentures 24 hours per day.

28 Papillary Hyperplasia: Treatment In cases of very early inflammatory papillary hyperplasia, removal of the denture may allow the erythema and edema to subside and the tissues may resume a more normal appearance. In cases of very early inflammatory papillary hyperplasia, removal of the denture may allow the erythema and edema to subside and the tissues may resume a more normal appearance. Lesions may show some improvement after topical or systemic antifungal therapy. Lesions may show some improvement after topical or systemic antifungal therapy. For advanced cases, excision of the lesion is treatment of choice prior to the fabrication of a new denture. For advanced cases, excision of the lesion is treatment of choice prior to the fabrication of a new denture. Partial and full-thickness surgical blade excision, curettage, electrosurgery and cryrosurgery have all been used with success in particular cases. Partial and full-thickness surgical blade excision, curettage, electrosurgery and cryrosurgery have all been used with success in particular cases.

29 Papillary Hyperplasia: Significance The lesion is not premalignant. The lesion is not premalignant. Following surgery the old denture can be lined with a temporary tissue conditioner that acts as a dressing and promotes healing. Following surgery the old denture can be lined with a temporary tissue conditioner that acts as a dressing and promotes healing. After healing, the patient should be encouraged to leave the new denture out at night and to practice good denture hygiene. After healing, the patient should be encouraged to leave the new denture out at night and to practice good denture hygiene.

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34 Peripheral Giant Cell Granuloma Giant Cell Epulis

35 Peripheral Giant Cell Granuloma: Cause This is a reactive lesion associated with chronic trauma or irritation. This is a reactive lesion associated with chronic trauma or irritation.

36 Peripheral Giant Cell Granuloma: Treatment Excision is the treatment of choice down to the underlying bone. Excision is the treatment of choice down to the underlying bone. Adjacent teeth should be carefully scaled to remove any source of irritation thus minimizing the risk of recurrence. Adjacent teeth should be carefully scaled to remove any source of irritation thus minimizing the risk of recurrence.

37 Peripheral Giant Cell Granuloma: Significance These lesions will remain indefinitely if not treated. These lesions will remain indefinitely if not treated. Remember, it is a reactive lesion similar in clinical appearance to the pyogenic granuloma. Remember, it is a reactive lesion similar in clinical appearance to the pyogenic granuloma.

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40 Peripheral Ossifying Fibroma Ossifying Fibroid Epulis Peripheral Fibroma with Calcification Calcifying Fibroblastic Granuloma

41 Peripheral Ossifying Fibroma (POF) The POF is a common reactive gingival growth. The POF is a common reactive gingival growth. Its pathogenesis is best describe as uncertain; some POFs have been suggested to develop from pyogenic granulomas. Its pathogenesis is best describe as uncertain; some POFs have been suggested to develop from pyogenic granulomas. It should be noted that POFs are not the same as peripheral odontogenic fibromas and they are not the soft tissue counterpart of the central ossifying fibroma. It should be noted that POFs are not the same as peripheral odontogenic fibromas and they are not the soft tissue counterpart of the central ossifying fibroma.

42 Peripheral Ossifying Fibroma (POF): Clinical Features This lesion occurs exclusively on the gingiva. This lesion occurs exclusively on the gingiva. It appears as a nodular mass usually arising from the interdental papilla area. It appears as a nodular mass usually arising from the interdental papilla area. It is red to pink in color and the surface is often ulcerated. It is red to pink in color and the surface is often ulcerated. POFs are more common in young adults and females. POFs are more common in young adults and females. There is a slight predilection for the maxilla and the anterior region of the jaws. There is a slight predilection for the maxilla and the anterior region of the jaws.

43 Peripheral Ossifying Fibroma: Treatment and Prognosis The treatment of choice is local surgical excision down to the periosteum to prevent recurrence. The treatment of choice is local surgical excision down to the periosteum to prevent recurrence. The adjacent teeth should be scaled to eliminate irritants such as calculus. The adjacent teeth should be scaled to eliminate irritants such as calculus. A recurrence rate of 16 % has been reported in the literature. A recurrence rate of 16 % has been reported in the literature.

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46 Hemangioma and Vascular Malformations

47 The term hemangioma has traditionally been used to describe a variety of developmental vascular anomalies. The term hemangioma has traditionally been used to describe a variety of developmental vascular anomalies. Currently, hemangiomas are considered to be benign tumors of infancy that are characterized by a rapid growth phase with endothelial cell proliferation, followed by a gradual involution. Most cannot be recognized at birth but make their appearance in most instances during the first 8 weeks of life. Currently, hemangiomas are considered to be benign tumors of infancy that are characterized by a rapid growth phase with endothelial cell proliferation, followed by a gradual involution. Most cannot be recognized at birth but make their appearance in most instances during the first 8 weeks of life. Vascular malformations are structural anomalies of blood vessels without endothelial proliferation and are present at birth and persist throughout life. Vascular malformations are structural anomalies of blood vessels without endothelial proliferation and are present at birth and persist throughout life.

48 Hemangioma Hemangiomas are the most common tumors of infancy. Hemangiomas are the most common tumors of infancy. They have a female gender predilection (3:1) and are most common in the White population. They have a female gender predilection (3:1) and are most common in the White population. 60% of the hemangiomas occur in the head and neck region with 80% of them occurring as single lesions. Multiple lesions may be part of a syndrome. 60% of the hemangiomas occur in the head and neck region with 80% of them occurring as single lesions. Multiple lesions may be part of a syndrome. About 50 % of all hemangiomas will show complete resolution by 5 years of age. About 50 % of all hemangiomas will show complete resolution by 5 years of age.

49 Vascular Malformations These lesions are present from birth and persist throughout life. These lesions are present from birth and persist throughout life. As with hemangiomas, these lesions tend to darken with age. As with hemangiomas, these lesions tend to darken with age. Low-flow venous malformations typically have a blue color and are easily compressible. Low-flow venous malformations typically have a blue color and are easily compressible. Arteriovenous malformations are high-flow lesions that result from persistent direct arterial and venous communications. A palpable thrill or bruit is often noticeable and the overlying skin typically feels warmer to the touch. Arteriovenous malformations are high-flow lesions that result from persistent direct arterial and venous communications. A palpable thrill or bruit is often noticeable and the overlying skin typically feels warmer to the touch.

50 Hemangioma and Vascular Malformations Because most hemangiomas undergo involution, management often consists of “watchful neglect”. For problematic hemangiomas, surgical resection or pharmacologic therapy may be indicated (systemic steroids or interferon-α-2a). Flashlamp- pulsed dye lasers can be effective in treating port wine stains. Because most hemangiomas undergo involution, management often consists of “watchful neglect”. For problematic hemangiomas, surgical resection or pharmacologic therapy may be indicated (systemic steroids or interferon-α-2a). Flashlamp- pulsed dye lasers can be effective in treating port wine stains. Management of vascular malformations depends on their size, location and associated complications. Sclerotherapy and surgical excision are the mainstays. Management of vascular malformations depends on their size, location and associated complications. Sclerotherapy and surgical excision are the mainstays. Treatment of arteriovenous malformations is more challenging and may involve surgical resection or embolization. Treatment of arteriovenous malformations is more challenging and may involve surgical resection or embolization. Central vascular malformations of the jaws are potentially dangerous lesions because of severe bleeding. Needle aspiration of any undiagnosed intrabony lesion before biopsy is a wise precaution to rule out these lesions. Central vascular malformations of the jaws are potentially dangerous lesions because of severe bleeding. Needle aspiration of any undiagnosed intrabony lesion before biopsy is a wise precaution to rule out these lesions.

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60 Sturge-Weber Angiomatosis This is a rare, non-hereditary developmental condition that is characterized by a harmartomatous vascular proliferation involving the tissue of the brain and face. This is a rare, non-hereditary developmental condition that is characterized by a harmartomatous vascular proliferation involving the tissue of the brain and face.

61 Sturge-Weber Angiomatosis: Clinical Features Patients are born with a dermal capillary vascular malformation of the face known as port wine stain or nevus flammeus because of its deep purple color. The port wine stain is usually unilateral. It should be noted that not all patients with port wine stain have Sturge-Weber. Only patients with involvement of the ophthalmic branch of the trigeminal nerve were at risk for the full condition. Patients are born with a dermal capillary vascular malformation of the face known as port wine stain or nevus flammeus because of its deep purple color. The port wine stain is usually unilateral. It should be noted that not all patients with port wine stain have Sturge-Weber. Only patients with involvement of the ophthalmic branch of the trigeminal nerve were at risk for the full condition.

62 Sturge-Weber Angiomatosis: Clinical Features Continued In addition to the port wine stain, affected individuals also have leptomeningeal angiomas that overlie the ipsilateral cerebral cortex. These angiomas are often associated with a convulsive disorder and may result in mental retardation or contralateral hemiplegia. In addition to the port wine stain, affected individuals also have leptomeningeal angiomas that overlie the ipsilateral cerebral cortex. These angiomas are often associated with a convulsive disorder and may result in mental retardation or contralateral hemiplegia. Skull radiographs may demonstrate “tranline” calcifications on the affected side. Skull radiographs may demonstrate “tranline” calcifications on the affected side. Ocular involvement includes: glaucoma and vascular malformations. Ocular involvement includes: glaucoma and vascular malformations. Intraoral involvement is common and manifests itself as vascular involvement of the affected tissue. Intraoral involvement is common and manifests itself as vascular involvement of the affected tissue.

63 Sturge-Weber Angiomatosis: Treatment and Prognosis Treatment and prognosis depends upon the severity of the case. Treatment and prognosis depends upon the severity of the case. Port wine nevi have been removed by laser. Port wine nevi have been removed by laser. Symptomatic leptomeningeal lesions are neurosurgical cases. Symptomatic leptomeningeal lesions are neurosurgical cases. Laser therapy has also been used for intraoral lesions; one should beware of surgery in the affected areas because of the possibility of severe hemorrhage. Laser therapy has also been used for intraoral lesions; one should beware of surgery in the affected areas because of the possibility of severe hemorrhage.

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68 Lymphangioma Lymphangiomas are benign, hamartomatous tumors of lymphatic vessels. Lymphangiomas are benign, hamartomatous tumors of lymphatic vessels. There are three types of lymphangiomas: There are three types of lymphangiomas: 1) lymphangioma simplex (capillary size vessels) 1) lymphangioma simplex (capillary size vessels) 2) cavernous lymphangioma (larger vessels) 2) cavernous lymphangioma (larger vessels) 3) cystic lymphangioma (cystic hygroma) with large macroscopic cystic spaces 3) cystic lymphangioma (cystic hygroma) with large macroscopic cystic spaces Lymphangiomas have a predilection for the head and neck with about half of all lesions present at birth. Lymphangiomas have a predilection for the head and neck with about half of all lesions present at birth.

69 Lymphangioma: Clinical Features Oral lymphangiomas occur most frequent on the anterior 2/3 of the tongue where they may produce macroglossia. Oral lymphangiomas occur most frequent on the anterior 2/3 of the tongue where they may produce macroglossia. They appear as a spongy, diffuse, painless mass which usually has a pebbly surface. Their appearance is one of a cluster of translucent vesicles having a red-blue hue. They appear as a spongy, diffuse, painless mass which usually has a pebbly surface. Their appearance is one of a cluster of translucent vesicles having a red-blue hue. Small lymphangiomas may appear on the alveolar ridges of neonates and are more common in African- Americans. These often occur bilaterally, have a male gender predilection and spontaneously resolve. Small lymphangiomas may appear on the alveolar ridges of neonates and are more common in African- Americans. These often occur bilaterally, have a male gender predilection and spontaneously resolve.

70 Lymphangioma: Treatment and Prognosis Treatment usually consists of surgical excision although total removal may not be possible in all cases. Treatment usually consists of surgical excision although total removal may not be possible in all cases. Lymphangiomas generally do not respond to sclerosing agents although recent studies indicate some success with OK-432. Lymphangiomas generally do not respond to sclerosing agents although recent studies indicate some success with OK-432. The prognosis is good for most patients although large tumors (cystic hygromas) may cause airway obstruction. The prognosis is good for most patients although large tumors (cystic hygromas) may cause airway obstruction.

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76 Giant Cell Fibroma

77 Giant Cell Fibroma: Clinical Features Giant cell fibromas usually present as asymptomatic, sessile or pedunculated nodules less than one centimeter in size. Giant cell fibromas usually present as asymptomatic, sessile or pedunculated nodules less than one centimeter in size. While they may have a smooth surface, they often have a papillary surface. While they may have a smooth surface, they often have a papillary surface. Sixty percent are diagnosed during the first three decades with a slight female gender predilection. Sixty percent are diagnosed during the first three decades with a slight female gender predilection. Fifty percent occur on the gingiva and the mandible is more commonly site of this lesion (2:1). Fifty percent occur on the gingiva and the mandible is more commonly site of this lesion (2:1).

78 Giant Cell Fibroma: Histological Features The mass is composed of vascular, loosely arranged fibrous connective tissue. The mass is composed of vascular, loosely arranged fibrous connective tissue. The hallmark of the lesion is the presence of large, stellate fibroblasts, which may contain several nuclei, within the fibrous connective tissue. The hallmark of the lesion is the presence of large, stellate fibroblasts, which may contain several nuclei, within the fibrous connective tissue. The covering epithelium is often thin and atropic and the rete ridges may be elongated and narrow. The covering epithelium is often thin and atropic and the rete ridges may be elongated and narrow. Lesions with a similar histological appearance found on the lingual mandibular gingiva in the region of the mandibular canine, have been called retrocuspid papillae. Lesions with a similar histological appearance found on the lingual mandibular gingiva in the region of the mandibular canine, have been called retrocuspid papillae.

79 Giant Cell Fibroma: Treatment and Prognosis Treatment consists of conservative surgical excision. Treatment consists of conservative surgical excision. If properly excised, the lesion rarely recurs. If properly excised, the lesion rarely recurs.

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83 Fibromatosis and Myofibromatosis

84 Fibromatosis and Myofibromatosis: Introduction Fibromatoses are a broad group of fibrous proliferations with a biological behavior ranging from benign to malignant. Fibromatoses are a broad group of fibrous proliferations with a biological behavior ranging from benign to malignant. Myofibromatosis is a similar but less aggressive proliferation of myofibroblasts. Myofibromatosis is a similar but less aggressive proliferation of myofibroblasts.

85 Fibromatosis and Myofibromatosis: Clinical Features Fibromatosis presents as a firm, painless mass, which may grow rapidly or slowly. Fibromatosis presents as a firm, painless mass, which may grow rapidly or slowly. Fibromatosis is more common in children and young adults with a mean age between 8 and 11 years. Fibromatosis is more common in children and young adults with a mean age between 8 and 11 years. The site predilection for fibromatosis is the paramandibular soft tissues. The site predilection for fibromatosis is the paramandibular soft tissues. These lesions can grow to a large size and can destroy bone. These lesions can grow to a large size and can destroy bone.

86 Fibromatosis and Myofibromatosis: Clinical Features Continued Myofibromatosis is seen most commonly in neonates and infants. Myofibromatosis is seen most commonly in neonates and infants. It commonly arises as a firm mass in the dermis or subcutaneous tissues of the head and neck. It commonly arises as a firm mass in the dermis or subcutaneous tissues of the head and neck. Cases of intraosseous myofibromatosis have been reported. Cases of intraosseous myofibromatosis have been reported. While these lesions are usually solitary, multiple lesions in the same patient have been reported. While these lesions are usually solitary, multiple lesions in the same patient have been reported.

87 Fibromatosis and Myofibromatosis: Treatment and Prognosis Fibromatosis is treated by wide surgical excision because these lesions are typically locally aggressive. Fibromatosis is treated by wide surgical excision because these lesions are typically locally aggressive. Oral/paraoral fibromatosis has approximately a 23 % recurrence rate. Oral/paraoral fibromatosis has approximately a 23 % recurrence rate. Myofibromatosis can be treated by local excision; cases of spontaneous regression have been reported. Myofibromatosis can be treated by local excision; cases of spontaneous regression have been reported. Multicentric lesions of myofibromatosis have a less favorable outcome. Multicentric lesions of myofibromatosis have a less favorable outcome.

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89 Fibromatosis

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91 Fibrous Histiocytoma Dermatofibroma Sclerosing Hemangioma Fibroxanthoma Nodular Subepidermal Fibrosis

92 Fibrous Histiocytoma Fibrous histiocytomas are a diverse group of tumors that exhibit both fibroblastic and histocytic differentation. Fibrous histiocytomas are a diverse group of tumors that exhibit both fibroblastic and histocytic differentation. They can occur anywhere in the body but those of the skin are most common and in this location are called dermatofibromas. They can occur anywhere in the body but those of the skin are most common and in this location are called dermatofibromas. They are uncommon in the oral/perioral region, with the buccal mucosa being the most common intraoral site. They are uncommon in the oral/perioral region, with the buccal mucosa being the most common intraoral site.

93 Fibrous Histiocytoma Most oral tumors are seen in middle-age and older adults. Most oral tumors are seen in middle-age and older adults. They typically appear as painless nodular masses and can range in size, with the deep tumors being larger. They typically appear as painless nodular masses and can range in size, with the deep tumors being larger. Local surgical excision is the treatment of choice and recurrence is uncommon. Local surgical excision is the treatment of choice and recurrence is uncommon.

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95 Fibrous Histiocytoma Fibrous Histiocytoma

96 Lipoma

97 Lipoma: Clinical Features and Cause Clinical Features: Lipomas appear as asymptomatic, slow-growing, well- circumscribed, yellow to yellow-white benign neoplasms of fat. Clinical Features: Lipomas appear as asymptomatic, slow-growing, well- circumscribed, yellow to yellow-white benign neoplasms of fat. While common elsewhere in the body, lipomas are uncommon intraorally. While common elsewhere in the body, lipomas are uncommon intraorally. The cause of lipomas is unknown. The cause of lipomas is unknown.

98 Lipoma: Treatment and Prognosis Excision is the treatment of choice for the oral lipoma. Excision is the treatment of choice for the oral lipoma. Oral lipomas seem to have a limited growth potential and recurrence is not expected after removal. Oral lipomas seem to have a limited growth potential and recurrence is not expected after removal.

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103 Traumatic Neuroma Amputation Neuroma

104 Traumatic Neuroma (Amputation Neuroma) This lesion is a reactive proliferation of neural tissue following transection or damage to the nerve bundle. This lesion is a reactive proliferation of neural tissue following transection or damage to the nerve bundle.

105 Traumatic Neuroma: Clinical Features These lesions generally present as smooth, non- ulcerated nodules. These lesions generally present as smooth, non- ulcerated nodules. Predilection sites include: mental foramen, tongue and lip. Predilection sites include: mental foramen, tongue and lip. There is often a history of trauma. There is often a history of trauma. They may produce a RL defect if bone is involved. They may produce a RL defect if bone is involved.

106 Traumatic Neuroma: Clinical Features Continued They may occur at any age but most cases occur in middle-aged adults and there is a slight female gender predilection. They may occur at any age but most cases occur in middle-aged adults and there is a slight female gender predilection. Only 25-33% are reported to be painful. If painful, the pain can be constant or intermittent and mild to severe. Only 25-33% are reported to be painful. If painful, the pain can be constant or intermittent and mild to severe. Lesions involving the mental foramen are most often painful especially if there is contact by a denture. Lesions involving the mental foramen are most often painful especially if there is contact by a denture.

107 Traumatic Neuroma: Histological Features Presents as a haphazard proliferation of mature, myelinated nerve bundles within a fibrous connective tissue stoma. Presents as a haphazard proliferation of mature, myelinated nerve bundles within a fibrous connective tissue stoma. The lesion may have an associated chronic inflammatory cell infiltrate. The lesion may have an associated chronic inflammatory cell infiltrate.

108 Traumatic Neuroma: Treatment and Prognosis Surgical excision is the treatment of choice with the excision to include a small portion of the involved nerve bundle. Surgical excision is the treatment of choice with the excision to include a small portion of the involved nerve bundle. Most lesions do not recur. Most lesions do not recur.

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114 Traumatic Neuroma

115 Palisaded Encapsulated Neuroma

116 Palisaded Encapsulated Neuroma: Clinical Features The etiology of this lesion is unknown but trauma has been suggested as a cause. The etiology of this lesion is unknown but trauma has been suggested as a cause. The lesion usually appears as a solitary, smooth, painless, dome-shaped papule or nodule. The lesion usually appears as a solitary, smooth, painless, dome-shaped papule or nodule. It occurs more commonly in adults and there is no gender predilection. It occurs more commonly in adults and there is no gender predilection. Common sites include the face, palate and lip. Common sites include the face, palate and lip.

117 Palisaded Encapsulated Neuroma: Histological Features As the name suggests the lesion is typically well- circumscribed and often encapsulated. As the name suggests the lesion is typically well- circumscribed and often encapsulated. The tumor consists of interlacing fascicles of spindle cells, which are probably Schwann cells. The tumor consists of interlacing fascicles of spindle cells, which are probably Schwann cells. The nuclei are wavy and pointed. The nuclei are wavy and pointed. While there is palisading there are no Verocay bodies of Antoni A tissue. While there is palisading there are no Verocay bodies of Antoni A tissue.

118 Palisaded Encapsulated Neuroma: Treatment and Prognosis Treatment consists of conservative local excision and recurrence is rare. Treatment consists of conservative local excision and recurrence is rare. This lesions is not associated with neurofibromatosis, multiple endocrine neoplasia syndrome and it does not undergo malignant change. This lesions is not associated with neurofibromatosis, multiple endocrine neoplasia syndrome and it does not undergo malignant change.

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121 Palisaded Encapsulated Neuroma

122 PEN VS Schwannoma

123 Neurilemoma Schwannoma

124 Neurilemoma (Schwannoma) This lesion is a benign neural neoplasm of Schwann cell origin. This lesion is a benign neural neoplasm of Schwann cell origin. It is a relatively uncommon lesion, although 25- 48 % of all cases occur in the head and neck region. It is a relatively uncommon lesion, although 25- 48 % of all cases occur in the head and neck region.

125 Neurilemoma: Clinical Features The neurilemoma is a slow-growing, encapsulated tumor associated with the nerve trunk. The neurilemoma is a slow-growing, encapsulated tumor associated with the nerve trunk. It is usually asymptomatic but pain may occur. It is usually asymptomatic but pain may occur. Most lesions occur in young to middle-aged adults. Most lesions occur in young to middle-aged adults. The tongue is the most common oral site. The lesion may occur in bone where it may cause expansion, radiolucency, pain or paresthesia. The tongue is the most common oral site. The lesion may occur in bone where it may cause expansion, radiolucency, pain or paresthesia.

126 Neurilemoma: Histological Features The neurilemoma is an encapsulated tumor composed of varying amounts of Antoni A and Antoni B tissue. The neurilemoma is an encapsulated tumor composed of varying amounts of Antoni A and Antoni B tissue. Antoni A tissue appears as streaming fascicles of spindle-shaped Schwann cells. These cells often form a palisaded arrangement around an acellular eosinophilic area known as a Verocay body. Antoni A tissue appears as streaming fascicles of spindle-shaped Schwann cells. These cells often form a palisaded arrangement around an acellular eosinophilic area known as a Verocay body.

127 Neurilemoma: Histological Features Continued Verocay bodies represent reduplicated basement membrane and cytoplasmic processes. Verocay bodies represent reduplicated basement membrane and cytoplasmic processes. Antoni B tissue is less cellular and less well organized. Antoni B tissue is less cellular and less well organized. Neurites can not be demonstrated within the mass. Neurites can not be demonstrated within the mass. Ancient neurilemomas Ancient neurilemomas

128 Neurilemoma: Treatment and Prognosis Surgical excision is the treatment of choice and the lesion should not recur. Surgical excision is the treatment of choice and the lesion should not recur. Extremely rare malignant transformation. Extremely rare malignant transformation.

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132 Neurofibroma/Neuro- fibromatosis

133 Neurofibroma/Neuro- fibromatosis: Clinical Features These lesions are soft, single or multiple, asymptomatic nodules covered by epithelium. These lesions are soft, single or multiple, asymptomatic nodules covered by epithelium. Intraorally, they may appear as the same color as or lighter in color than the surrounding mucosa. Intraorally, they may appear as the same color as or lighter in color than the surrounding mucosa. Most frequently they are found on the tongue, buccal mucosa and vestibule but may occur anywhere. Most frequently they are found on the tongue, buccal mucosa and vestibule but may occur anywhere. They may occur at any age and there is no gender predilection. They may occur at any age and there is no gender predilection.

134 Neurofibroma/Neurofibromatosis: Cause Unknown for the solitary neurofibromas while neurofibromatosis is an autosomal dominant disease entity. Unknown for the solitary neurofibromas while neurofibromatosis is an autosomal dominant disease entity. Approximately 50 % of cases of neurofibromatosis present no family history and are considered the result of spontaneous mutation. Approximately 50 % of cases of neurofibromatosis present no family history and are considered the result of spontaneous mutation. Neurofibromatosis is associated with NF1 and NF2 genes. Neurofibromatosis is associated with NF1 and NF2 genes.

135 Neurofibroma/Neurofibromatosis: Treatment and Prognosis The treatment of solitary neurofibromas is excision and recurrence is not expected. The treatment of solitary neurofibromas is excision and recurrence is not expected. Multiple neurofibromas should suggest neurofibromatosis (von Recklinghausen disease), which consists of multiple neurofibromas with malignant potential, cafè au lait spots, optic gliomas, Lisch nodules (iris hamartomas) and bony lesions as outlined in the next slide. Multiple neurofibromas should suggest neurofibromatosis (von Recklinghausen disease), which consists of multiple neurofibromas with malignant potential, cafè au lait spots, optic gliomas, Lisch nodules (iris hamartomas) and bony lesions as outlined in the next slide.

136 Box 12-1 Diagnostic Criteria for Neurofibromatosis Type I The diagnostic criteria are met if a patient has two or more of the following features: The diagnostic criteria are met if a patient has two or more of the following features: 1.Six or more café au lait macules over 5 mm in greatest diameter in prepubertal persons and over 15 mm in greatest diameter in postpubertal persons 1.Six or more café au lait macules over 5 mm in greatest diameter in prepubertal persons and over 15 mm in greatest diameter in postpubertal persons 2.Two or more neurofibromas of any type or one plexiform neurofibroma 2.Two or more neurofibromas of any type or one plexiform neurofibroma 3.Freckling in the axillary or inguinal regions 3.Freckling in the axillary or inguinal regions 4.Optic glioma 4.Optic glioma 5.Two or more Lisch nodules (iris hamartomas) 5.Two or more Lisch nodules (iris hamartomas) 6.A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis 6.A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis 7.A first-degree relative (parent, sibling, or offspring) with neurofibromatosis type I, based on the above criteria 7.A first-degree relative (parent, sibling, or offspring) with neurofibromatosis type I, based on the above criteria

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147 Multiple Endocrine Neoplasia Type 2B (MEN Type 2B or III) Multiple Endocrine Neoplasia Type III Multiple Mucosal Neuroma Syndrome

148 MEN 2B: Clinical Features Multiple neuromas may be associated with this syndrome and are more common on the lips, tongue and buccal mucosa. These neuromas are usually the first sign of the condition. Multiple neuromas may be associated with this syndrome and are more common on the lips, tongue and buccal mucosa. These neuromas are usually the first sign of the condition. The oral neuromas present as soft, painless papules or nodules. The oral neuromas present as soft, painless papules or nodules. Patients usually have a marfanoid body build, characterized by thin, elongated limbs with muscle wasting. While the face is typically narrow, the lips are usually thick because of the proliferation of nerve bundles. Of greatest significance is the development of medullary carcinoma of the thyroid. These patients may also develop a pheochromocytoma and an associated hypertensive problem. Patients usually have a marfanoid body build, characterized by thin, elongated limbs with muscle wasting. While the face is typically narrow, the lips are usually thick because of the proliferation of nerve bundles. Of greatest significance is the development of medullary carcinoma of the thyroid. These patients may also develop a pheochromocytoma and an associated hypertensive problem.

149 MEN 2B: Cause and Treatment The exact cause of MEN 2B is unknown although it has an autosomal dominant pattern of inheritance. The exact cause of MEN 2B is unknown although it has an autosomal dominant pattern of inheritance. While the oral neuromas may be excised, treatment focuses on the prevention of the medullary carcinoma of the thyroid and the observation of the patient for the development of the pheochromocytoma and its accompanying hypertension. While the oral neuromas may be excised, treatment focuses on the prevention of the medullary carcinoma of the thyroid and the observation of the patient for the development of the pheochromocytoma and its accompanying hypertension.

150 MEN 2B: Significance Given the serious nature of the medullary thyroid carcinoma, some investigators advocate prophylactic removal of the thyroid gland at an early age as the cancer is almost certain to occur. Given the serious nature of the medullary thyroid carcinoma, some investigators advocate prophylactic removal of the thyroid gland at an early age as the cancer is almost certain to occur. The hypertension associated with the development of a pheochromocytoma may be life-threatening. The hypertension associated with the development of a pheochromocytoma may be life-threatening.

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154 Mucosal Neuroma of MEN III

155 Neural Tumors: Comparative Features

156 Granular Cell Tumor

157 Granular Cell Tumor: Clinical Features These lesion typically appear as painless, elevated tumescences covered by an intact epithelium. These lesion typically appear as painless, elevated tumescences covered by an intact epithelium. Their color may be the same as or lighter than the surrounding tissue. Their color may be the same as or lighter than the surrounding tissue. There is a strong predilection for the dorsum of the tongue but may be found anywhere. There is a strong predilection for the dorsum of the tongue but may be found anywhere. Granular cell tumors are rare in children and there is a 2:1 female gender predilection. Granular cell tumors are rare in children and there is a 2:1 female gender predilection.

158 Granular Cell Tumor The cause of the granular cell tumor is unknown and the cell of origin is undetermined. The cause of the granular cell tumor is unknown and the cell of origin is undetermined. Originally, it was considered to be of skeletal muscle origin but current research suggests origin from Schwann cells or neuorendocrine cells. Originally, it was considered to be of skeletal muscle origin but current research suggests origin from Schwann cells or neuorendocrine cells. Treatment consists of surgical excision and the lesion does not recur. Treatment consists of surgical excision and the lesion does not recur. The lesion must be differentiated from other lesions of the tongue particularly squamous cell carcinoma as a superficial biopsy of a granular cell tumor with PEH may be confused with this cancer. The lesion must be differentiated from other lesions of the tongue particularly squamous cell carcinoma as a superficial biopsy of a granular cell tumor with PEH may be confused with this cancer.

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165 Congenital Epulis Congenital Epulis of the Newborn Congenital Granular Cell Lesion

166 Congenital Epulis: Clinical Features and Cause The congenital epulis generally appears as a firm pedunculated or sessile mass attached to the gingiva of infants. The congenital epulis generally appears as a firm pedunculated or sessile mass attached to the gingiva of infants. It may be the same color as or lighter than the surrounding tissue. It may be the same color as or lighter than the surrounding tissue. The cause of the congenital epulis is unknown. The cause of the congenital epulis is unknown.

167 Congenital Epulis: Treatment and Prognosis Surgical excision is the treatment of choice. Some lesions undergo spontaneous regression. Surgical excision is the treatment of choice. Some lesions undergo spontaneous regression. The lesion is not expected to recur. The lesion is not expected to recur.

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171 Osseous and Cartilaginous Choristomas

172 A choristoma is a tumor-like growth of microscopically normal tissue in an abnormal location. A choristoma is a tumor-like growth of microscopically normal tissue in an abnormal location. Choristomas of the mouth have included gastric mucosa, glial tissue and masses of sebaceous glands. Choristomas of the mouth have included gastric mucosa, glial tissue and masses of sebaceous glands. The most frequently observed choristomas of the oral cavity are those composed of bone or cartilage or both. The most frequently observed choristomas of the oral cavity are those composed of bone or cartilage or both.

173 Osseous and Cartilaginous Choristomas: Clinical Features These lesions have a marked predilection for the tongue (approximately 85 % of the cases). These lesions have a marked predilection for the tongue (approximately 85 % of the cases). Most occur near the foramen cecum. Most occur near the foramen cecum. These choristomas usually present as firm, smooth, sessile or pedunculated nodules. These choristomas usually present as firm, smooth, sessile or pedunculated nodules. Occasionally patients will complain of gagging or dysphagia. Occasionally patients will complain of gagging or dysphagia. More than 70 % of these choristomas have been reported in women. More than 70 % of these choristomas have been reported in women.

174 Osseous and Cartilaginous Choristomas:Treatment & Prognosis Local surgical excision is the treatment of choice. Local surgical excision is the treatment of choice. Recurrence has not been reported. Recurrence has not been reported.

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177 Soft Tissue Sarcomas Soft tissue sarcomas are rare malignant tumors of the oral and maxillofacial region accounting for less than 1% of the cancers in this area.

178 Fibrosarcoma Fibrosarcomas are malignant tumors of fibroblasts. Fibrosarcomas are malignant tumors of fibroblasts. They were once considered the most common soft tissue sarcoma but are considered uncommon today (and only 10% of them occur in the head and neck region). They were once considered the most common soft tissue sarcoma but are considered uncommon today (and only 10% of them occur in the head and neck region).

179 Fibrosarcoma: Clinical Features Fibrosarcomas most often present as slow- growing masses which may reach considerable size before producing pain. Fibrosarcomas most often present as slow- growing masses which may reach considerable size before producing pain. They can occur anywhere in the oral region. A number of cases have been reported in the nose and paranasal sinuses where obstructive symptoms are the chief complaint. They can occur anywhere in the oral region. A number of cases have been reported in the nose and paranasal sinuses where obstructive symptoms are the chief complaint. They can occur at any age but are more common in children and young adults. They can occur at any age but are more common in children and young adults.

180 Fibrosarcoma: Treatment and Prognosis Surgical excision with wide margins is the treatment of choice. Surgical excision with wide margins is the treatment of choice. The 5-year survival rates range from 40-70 %. The 5-year survival rates range from 40-70 %.

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186 Malignant Fibrous Histiocytoma This sarcoma, which has both fibroblastic and histiocytic features, is now considered to be the most common soft tissue sarcoma in adults. This sarcoma, which has both fibroblastic and histiocytic features, is now considered to be the most common soft tissue sarcoma in adults.

187 Malignant Fibrous Histiocytoma: Clinical Features This is primarily a tumor of older age groups. This is primarily a tumor of older age groups. The most common complaint is an expanding mass that may or may not be ulcerated or painful. The most common complaint is an expanding mass that may or may not be ulcerated or painful. Tumors of the nasal cavity or paranasal sinuses usually produce obstructive symptoms. Tumors of the nasal cavity or paranasal sinuses usually produce obstructive symptoms.

188 Malignant Fibrous Histiocytoma: Treatment and Prognosis This is an aggressive tumor that typically requires radical surgical resection. This is an aggressive tumor that typically requires radical surgical resection. Approximately 40 % of patients have local recurrences with a similar number developing metastases within 2 years of initial diagnosis. Approximately 40 % of patients have local recurrences with a similar number developing metastases within 2 years of initial diagnosis. The survival rate for patients with oral tumors seems to be worse that for those with the tumor at other body sites. The survival rate for patients with oral tumors seems to be worse that for those with the tumor at other body sites.

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190 Metastases to the Oral Soft Tissues This is an uncommon but very serious event. This is an uncommon but very serious event. Most common site is the gingiva followed by the tongue. Most common site is the gingiva followed by the tongue. Lesions usually present as nodular masses often resembling hyperplastic or reactive growths. Lesions usually present as nodular masses often resembling hyperplastic or reactive growths. Occasional lesions show ulceration and the adjacent teeth may become loose. Occasional lesions show ulceration and the adjacent teeth may become loose.

191 Metastases to the Oral Soft Tissues This occurs more commonly in middle-aged and older adults. This occurs more commonly in middle-aged and older adults. There is a male gender predilection. There is a male gender predilection. In males, the most common sites of the primary tumors are: the lung, kidney and skin (melanoma). Prostate cancer has an affinity for bone rather than soft tissue. In males, the most common sites of the primary tumors are: the lung, kidney and skin (melanoma). Prostate cancer has an affinity for bone rather than soft tissue.

192 Metastases to the Oral Soft Tissues In females, the most common sites for the primary tumors are the: breast, genital organs, lung, bone and kidney. In females, the most common sites for the primary tumors are the: breast, genital organs, lung, bone and kidney. In most cases the primary tumor is know before the oral metastases are discovered indicating we need a good medical history. In most cases the primary tumor is know before the oral metastases are discovered indicating we need a good medical history. The metastases should resemble the primary tumor microscopically and this has been helpful in detecting undiscovered tumors. The metastases should resemble the primary tumor microscopically and this has been helpful in detecting undiscovered tumors. Prognosis is generally poor. The management of the oral lesion is usually only palliative. Prognosis is generally poor. The management of the oral lesion is usually only palliative.

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