2. ALAA AL- BAYOUK,RMT,MD,PhD.
CONSULTANT & HEAD OF PATHOLOGY DEPT.
NASSER MEDICAL COMPLEX

3. Case presentation

4. Personal data
M.SH
Female
36 years old

5. Clinical
Huge abdominal mass
Intestinal obstruction

6. Radiologcal USG CT - abdomen & pelvis Calcular GB
Big mid & lower abdomen mesenteric lesion
with mass effect suggesting sclerosing mesenteritis associated with inflammatory process without sizable collection.

8. Surgical Urgent exploratory laparotomy
Transverse colon mass with internal abdominal wall involvement.
En-bloc resection with extended right hemicolectomy

9. Pathology
Gross
Microscopy

10. Gross Muscular fascia 20x10 cm, Grayish, white glistening
Colonic segment 100 cm /large mass 25x10 cm
Adherent to bowel wall / serosa, not mucosal
Muscular fascia 20x10 cm,
Infiltrative borders
Grayish, white glistening
Firm homogenous
Gritty sensation CS
No hemorrhage
No necrosis

14. Microscopy Spindled shaped fibroblasts, uniform Infiltrative margins
Abundant collagen
Myxoid matrix
Vessels, delicate , compressed
Lymphocytic infiltration
No/ occ. mitosis
Mild atypia
No necrosis
Serosa/ infiltation / Mucosa- LP (intact)

22. Approach
Tumor ? Type ? B9
Malignant
Primary
Secondary
Metastatic

23. Histology
Epithelial
Mesenchymal / Ct.
Muscle
Nervous

25. Provisional diagnosis
Fibromatosis
- superficial
- Deep (DESMOID TUMOR)

26. Differential Diagnosis
GIST strong CD117+, CD34+
Fibrosarcoma
Low grade fibromyxoid sarcoma
Leiomyoma
Idiopathic retroperitoneal fibrosis
Neurofibroma
Shwannoma
Sclerosing omentitis

27. Final Diagnosis MESENTERIC FIBROMATOSIS WITH INTESTINAL INVOLVEMENT.
TRANSVERSE COLON MASS WITH ABDOMINAL WALL INVOLVEMENT, BIOPSY:
MESENTERIC FIBROMATOSIS WITH INTESTINAL INVOLVEMENT.
(INTRAABDOMINAL DESMOID TUMOR) .
NO SIGNS OF MALIGNANCY .

28. Fibromatosis Abdominal Extra-abdominal Intra-abdominal
Abdominal wall of women during or after pregnancy
May see with cesarean section scar
Extra-abdominal
Outside abdomen and abdominal wall, usually in muscles of shoulder, chest wall, back and thigh, equal gender frequencies
Intra-abdominal
Mesenteric, pelvic or retroperitoneal locations,
Often post-surgical

29. Fibromatosis
Most patients present with asymptomatic abdominal mass which is large, measuring 10 cm or more.
MF resembles gastrointestinal stromal tumors (GIST) that are mesenchymal neoplasms of the digestive tract and show a varied malignant potential.

30. Mesenteric fibromatosis (MF)
Definition / General
MF or intra-abdominal desmoid tumor is a rare proliferative disease affecting the mesentery.
MF is a locally aggressive tumor that lacks metastatic potential, but the local recurrence is common.

31. Mesenteric fibromatosis (MF)
Epidemiology
The most common primary tumor of mesentery ~ 8 % of all fibromatosis
Incidence of per million population, less common than superficial fibromatosis
Most cases are sporadic

32. Mesenteric fibromatosis (MF)
Epidemiology
May be familial (associated with Gardner's syndrome / FAP syndrome
Clin Gastroenterol Hepatol 2008;6:215
Hyperestrogenic states

33. Mesenteric fibromatosis (MF)
Epidemiology
Related to trauma
In a patient with a genetic predisposition, tissue injury like previous operation likely to be the cause

34. Fibromatosis Clinical features
Usually ages years, may be painful
Seen in breast, colon , small bowel
Common sites in children are head and neck
May be fatal due to local effects, particularly in head and neck

35. Fibromatosis Treatment
Excision with wide margins (Ann Surg Oncol 2009;16:1642)
Inadequate excision may cause recurrence, less recurrence in abdominal wall than elsewhere but may recur times
May stop growing if stop excising, some recommend watchful waiting if asymptomatic or not growing
(Expert Rev Anticancer Ther 2009;9:525)

37. Fibromatosis Treatment
May respond to chemotherapy (J Clin Oncol2007;25:501)
COX2 inhibitors (Urology 2007;70:591.e3),
Imatinib / Gleevec (J Cancer Res Clin Oncol 2007;133:533),
NSAIDs (World J Surg Oncol 2008;6:17),
Radiation (Am J Clin Oncol 2005;28:211),
Ramoxifen
Watchful waiting (Eur J Surg Oncol 2008;34:462)

38. Fibromatosis Clinical features
Stage: based on size, symptoms and complications
Dis Colon Rectum 2008;51:897
Prognostic factors: age, tumor size, tumor site
J Clin Oncol 2011;29:3553)

39. Case reports
27 year old woman with post-traumatic paraspinal mass (World J Surg Oncol 2008;6:28)
29 year old woman with tumor of abdominal wall (Radiology 2005;236:81)
29 year old woman with large desmoid tumor of the anterior abdominal wall (Internet J of Surg 2007;10:2)
30 year old man with aggressive mesenteric tumor that responded to chemotherapy (Jpn J Clin Oncol 2008;38:222)
43 year old woman with tumor of retroperitoneal space (World J Surg Oncol 2004;2:33)

40. Conclusion
Treatment of mesenteric fibromatosis is a multidisiplinary approach

41. Conclusion
Non- surgical treatment resulted in diverse & unpredictable outcome and it is considered to be an opportunity in patients with unrespectable lesions or for adjuvant therapy.

42. Conclusion
Surgery has a key role in the management and radical resection with clear margins is the principal treatment of this tumor entity.

44. Take home message:
Although intra-abdominal lesions with spindle cell morphology are relatively rare, and similarities in their clinical data, radiological and histopathological appearances may lead to misdiagnosis, it is also true that tumor diagnosis based on IHC staining or traditional criteria alone are not specific enough.

45. Take home message:
Intra abdominal fibromatosis is benign and exclusively locally aggressive, where as GISTs, are malignant and potentially capable of leading to distant metastasis.