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ALAA AL- BAYOUK,RMT,MD,PhD .

ALAA AL- BAYOUK,RMT,MD,PhD. CONSULTANT & HEAD OF PATHOLOGY DEPT. NASSER MEDICAL COMPLEX. Case presentation. Personal data. M.SH Female 36 years old. Clinical. Huge abdominal mass Intestinal obstruction. Radiologcal. USG CT - abdomen & pelvis Calcular GB

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ALAA AL- BAYOUK,RMT,MD,PhD .

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

  2. Case presentation

  3. Personal data • M.SH • Female • 36 years old

  4. Clinical • Huge abdominal mass • Intestinal obstruction

  5. 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.

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

  7. Pathology • Gross • Microscopy

  8. Gross • 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

  9. 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)

  10. Approach • Tumor ? Type ? • B9 • Malignant • Primary • Secondary • Metastatic

  11. Histology • Epithelial • Mesenchymal / Ct. • Muscle • Nervous

  12. Provisional diagnosis • Fibromatosis - superficial - Deep (DESMOID TUMOR)

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

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

  15. Fibromatosis • 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

  16. 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.

  17. 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.

  18. Mesenteric fibromatosis (MF) Epidemiology • The most common primary tumor of mesentery ~ 8 % of all fibromatosis • Incidence of 2 - 4 per million population, less common than superficial fibromatosis • Most cases are sporadic

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

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

  21. Fibromatosis Clinical features • Usually ages 15 - 39 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

  22. 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 5 - 6 times • May stop growing if stop excising, some recommend watchful waiting if asymptomatic or not growing (Expert Rev Anticancer Ther 2009;9:525)

  23. 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)

  24. 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)

  25. 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)

  26. Conclusion • Treatment of mesenteric fibromatosis is a multidisiplinary approach

  27. 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.

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

  29. 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.

  30. 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.

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