Mesenteric Fibromatosis: By Saud
Case Report and Literature
A. Al Jadaan
ESENTERIC FIBROMATOSIS is a rare benign intraabdominal tumor. It can infiltrate the surrounding structures and tends to recur locally, but it never metastasizes. Most of the reported cases have been in association with Gardener’s syndrome, previous trauma, and prolonged estrogen intake, but mesenteric fibromatosis can occur as a primary condition (primary mesenteric fibromatosis) in the absence of those predisposing factors. We report a case of mesenteric fibromatosis in a young girl. CASE
A 32-month-old girl presented to our hospital with a l-month history of abdominal pain associated with anorexia, but this patient exhibited no noticeable weight loss or other symptoms. She was the product of a normal pregnancy during which time there was no history of maternal drug ingestion. Her family history was unremarkable. On physical examination she was noted to have a left upper quadrant mass, which was mobile; there were no other significant findings. A computed tomography (CT) scan of the abdomen showed a mesenteric mass with a consistency suggesting thick mutinous or proteinaceous material (Fig 1). It measured 7 X 5.6 cm. The liver, spleen, and pancreas appeared normal. There were no other significant findings. At laparotomy a solid mass measuring 9 X 7 X 10 cm was identified at the root of the jejunal mesentery in close association with the superior mesenteric and the middle colic vessels (Fig 2). The mesentery contained several large lymph nodes. A small amount of free peritoneal fluid was present. The mass was totally excised without apparent
Mesentericfibromatosis is a rare, benign fibrous lesion found in the bowel mesentery or the retroperitoneum. Its biological behavior is intermediate between benign fibrous tissue proliferation and fibrosarcoma. Fibromatosis characteristically is locally invasive and tends to recur but does not metastasize. Most reported cases have been in older individuals, and there is a frequent association with familial polyposis coli, previous trauma, and hormonal imbalance. The authors report a case of mesenteric fibromatosis in a 32-month-old girl with a l-month history of abdominal pain who was discovered to
have an abdominal mass. the mass was excised. The diagnosis of fibromatosis. dren, as in adults, presents surgeon. J Pediatr Surg 34:1130-l Saunders Company. INDEX tumor.
After appropriate investigations, pathology report confirmed the Mesenteric fibromatosis in chila management challenge for the 132.
interference with the blood supply to the bowel. However, clinically significant bowel ischemia did occur, and the patient required a second laparotomy 5 days later. The ischemic injury seemed to be secondary to venous obstruction. It was necessary to resect a segment of small bowel measuring 50 cm in length. A primary anastomosis was performed. The patient did well and was discharged from the hospital 1 week after the second laparotomy. The final tissue diagnosis showed benign-looking spindle-shaped fibroblasts with elongated nuclei. The fibroblasts were separated by a very loose, small amount of collagen with abundant myxoid deposition. Absence of pleomorphism and mitosis were also noted (Fig 3), which was compatible with mesenteric fibromatosis with the lymph nodes showing reactive hyperplasia. A small sample of peritoneal fluid was free of malignant cells. Six months after the resection, a repeat CT scan of the abdomen showed no evidence of residual or recurrent tumor. The patient continues to do well. DISCUSSION
Fibromatoses are a broad group of benign fibrous tissue proliferations of similar microscopic appearance that are intermediate in their biological behavior between
From the Department of Surgery, Division of Pediatric Surgery, King Fahad National Guard Hospital, Riyadh, Saudi Arabia. Presented at the 30th Annual Meeting of the Canadian Association of Paediatrk Surgeons, Toronto. Ontario, Canada, September 25-28, 1998. Address reprint requests to Dr Saud Al Jadaan, Department of Surgery Dwision of Pediatric Surgery King Fahad National Guard Hospital, PO Box 22490, Rlyadh 11426, Saudi Arabia. Copyright 0 1999 by WB. Saunders Company 0022-3468/99/3407-0022$03.00/O
large soft tissue
No 7 (July),
1999: pp 1130-1132
benign fibrous lesions and fibrosarcoma. They tend to be locally invasive and to recur locally, but they do not metastasize.’ They are divided into deep and superficial types. Deep fibromatoses (also known as desmoid tumors) tend to be more aggressive in their behavior than the superficial ones.’ Most of the reported cases have been in the adult population. There is a strong association with familial polyposis, especially Gardner’s syndrome”-7; the process also has been reported after abdominal surgery.Z,5,7prolonged estrogen intake, pregnancy,2*3 and in a Crohn’s disease patient, with previous surgical intervention.8 Roger et al9 reported an association between mesenteric fibromatosis and melorheostosis (a benign, congenital disorder of bone and soft tissue). Mesenteric fibromatosis may occur as an isolated
Fig 2. mesentery.
Fig 3. Section shows fibroblasts separated by bands of collagen with abundant myxoid deposition. (H&E, original magnification x250.)
condition.lO-‘” The mesentery of the small bowel is the most common site, but it also has been identified in the mesocolon and the omenturn.? Yannopoulos and StoutI reported two cases of mesenteric fibromatosis in children; one child was 2 years old, and the other was 1%months old. Mecrow et all4 reported this condition in a 7-year-old boy who presented with acute ascites. Other reports appear to be restricted to the adult population. Solitary intestinal fibromatosis (which arises from the intestinal wall rather than the bowel mesentery) has been reported as affecting the bowel wall in neonates and causing intestinal obstruction or perforation. 15.16Mesenteric fibromatosis usually is heralded clinically by an abdominal mass, which often is associated with pain and weight loss. The treatment of choice is surgical removal, and this often requires resection of a segment of bowel and the subjacent mesentery affected by the proliferative abnormality. Complete resection may not be possible because of the large size of the lesion or intimate involvement with vital structures. In patients with unresectable tumors or recurrences, other nonsurgical treatments including the nonsteroidal antiinflammatory agent, sulindac (Clinoril); estrogen receptor antagonist, tamoxifen (Nolvadex); chemotherapy with dactinomycin, vincristine, and cyclophosphamide; and radiotherapy have been utilized, individually or in combinations, with variable success.3.J.17
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