2Case Presentation of Retroperitoneal Mass By : Dr. Khalid Jamal Hamdi
3History MRN:517400 (Hera Hospital) Saad is 43 y/o saudi male patient Presented to ERMain complaint:left lower quadrant pain x 2days associated withLower back pain
4HistoryHPI: not known to have any medical illness before presented to ER C/O LLQ pain x 2 days ,which started gradually, dull aching ,mild ,radiating to the back ,not aggrevated ,relieved by NSAID.The patient had a previous similar attacks during the last 6 months but he didn’t seek medical advise
5History Review of systems: No H/o : Dysuria ,nausea ,vomiting ,change inBowel habit, fever.No H/o : traumaother review of systems were unremarkablePast M Hx: unremarkable.Past surgical Hx: appendectomy 14 y ago.Social Hx :Smoker 2 packs /daymarried ,had 5 kidsAllergy: No non allergies to food or medication
6Examination Vitals : HR:60 bpm ,RR: 18 BP:130/90 , T : 36.7 General: conscious ,alert ,orientednot in pain , No Jaundice or pallorNo cyanosis , ( PI: 2/10 )Chest : clear ,EAE bilateralyCVS: S1 + S2 + 0
7Examination Abdomen: not distended, scar for appendectomy Soft,lax , no tenderness , no organomegalyNo palpable massAudiable bowel soundCNS: Normal
8Investigations CBC, Hgb:16 g/dl ,WBC:4.0 ,Plt:220 Chemistry: Normal Abdominal U/S :done in a private clinicshows huge echogenic well defined homogenously solid mass (11.7x8.4 cm)at retrovesical pouch (?? Colonic mass)
9Investigations CT abdomen &pelvis (triple contrast): shows evidence of well defined soft tissue mass in the pelvic cavity about (10.5x8.5cm)seen at the area behind the urinary bladder more to the left side at the level of rectosigmoid with pedicle toward the sacrum.the mass displace the UB ant. and Compressing the area of rectosigmoid
10Investigations CT abdomen &pelvis (triple contrast): Cont’ No bony erosionsOther abdominal organs are normalNo free fluidColonoscopy: Normal study
11differential diagnosis of retroperitoneal mass ( in general ) 1. Primary Tumors of Retroperitoneuma. Sarcomasb. Neuroendocrine tumors - neurofibroma, ganglioneuroma, Schwannomas, …etc.c. Lymphomasd. Diffuse retroperitoneal carcinomas (undifferentiated and metastatic origen)E. kidney,adrenal and ureteric tumors
12differential diagnosis of retroperitoneal mass 2. Retroperitoenal Fibrosisa. Primary (idiopathic) - about 70% of casesb. Secondary (listed below)Drugs - methysergide, methyldopa, ergot alkyloidsPeriarteritis - usually with aortic aneurysmMalignant tumorsRadiational fibrosisInfection - intrabdominal, gonorrhea, abscessLymphangitisRetroperitoneal Hemorrhage (often after invasive proceedure)Connective Tissue / Autoimmune Disease – systemic sclerosis, systemic lupusGranulomatous Disease - tuberculosis, sarcoidosis
13ManagementPlan :Exploratory Lap, total excision of retroperitoneal massFinding intra operatively:huge retroperitoneal mass approximately10x10 cmadherent to Lt ureter ,Lt Iliac vessels,presacral fascia
17Histopathology.Histological features of benign peripheral nerve sheath tumor,neurilemoma(cellular schwannoma)
18and belong to the category of SchwannomasSchwannomas ( neurilemomas).are usually benign tumors arising from Schwann cells of the peripheral nerve sheath.and belong to the category ofneural sheath tumors
19SchwannomasMostschwannomas are benign, although malignant cases are known to occur,especially when there is association withVon Recklinghausen‘s disease,rarely occur in the retroperitoneum, comprising 0.5% to 5% of all schwannomas.
20SchwannomasTheir usual location is the head, neck, the flexor surfaces of the extremities and the posterior mediastinum or the retroperitoneumThey can be found in any nerve trunk,except for ?? ,Bastounis E, Asimacopoulos PJ, Picoulis E, Leppaniemi AK, AggourasD, Papakonstandinou K, Papalambros E: Benign retroperitonealneural sheath tumors in patients without von Recklinghausen'sdisease. Scand J Urol Nephrol
22Schwannomas Diagnosis in the retroperitoneal position is difficult, and a large and deeply situated tumor is usuallypresent before patients have any symptomssymptomsare vague and nonspecific,such as vague abdominal painand dull ache.Atypical presentations: are very rare and includeflank pain and hematuria, headache, and secondaryhypertension and recurrent renal colic pain.
23Schwannomas gross appearance, schwannomas are usually solitary, well circumscribed,firm, smooth-surfaced tumorsHistologically, schwannomas consist of compactcellular lesions (Antoni type A tissue) and loose,hypocellular myxoid lesions with microcystic spaces (Antonitype B tissue).,The hallmark pattern of the benign variantsis an alternation of these Antoni A and B areas,with a diffuse positivity for S100 protein in the cytoplasm of thetumor cells.
28Schwannomas Malignant degeneration of schwannomas is extremely rare, but when present, they act as high-grade sarcomaswith a high likelihood of producing local recurrence and distant metastasis.
29Schwannomas Radiological studies are fundamental in the diagnostic evaluation.Computed tomography(CT) scans typically show welldefinedlow or mixed attenuation with cystic necrotic centralareas.Cystic changes occur more commonly in retroperitoneal schwannomas (up to 66%) than in other retroperitoneal tumors.
30SchwannomasOther degenerative changes, such as calcification, hemorrhage, and hyalinization, can also be present
31Schwannomas CT-guided core biopsy and fine needle aspiration have been founded to be unreliable for the diagnosis of retroperitoneal schwannoma.Risk of hemorrhage, infection, and tumor seeding; thus,many authors do not recommend CT-guided biopsy.
32Schwannomas Management : surgical resection is the only accurate approachfor pathologic evaluation to enable diagnosis of retroperitonealschwannoma.
33Schwannomas Recent advances in laparoscopic instruments and skills have made laparoscopy an excellentapproach for biopsy and even surgical resection of benign retroperitoneal tumors
34neural sheath tumors is complete excision, SchwannomasEven though the best management of retroperitonealneural sheath tumors is complete excision,considerablecontroversy exists over negative soft tissue margins.
35Schwannomas The argument here is that the morbidity associated with resection of adjacent tissuewould not be justified in the treatment of a benign lesionprognosis for retroperitoneal benignschwannoma is extremely good
36Schwannomas Followup:malignant transformations have been reported, usually associated with von Recklinghausen‘sdisease.(*) There are a few reported cases in whichmetastases occurred after resection of a histologically benign schwannoma.??Therefore, it is suggested that carefulmonitoring is necessary after removal of benign retroperitoneal SchwannomasOhigashi T, Nonaka S, Nakanoma T, Ueno M, Deguchi N. Laparoscopictreatment of retroperitoneal benign schwannoma. Int J Urol