Mesenteric fibromatosis: Case report and literature review

Mesenteric fibromatosis: Case report and literature review

Mesenteric Fibromatosis: By Saud Case Report and Literature A. Al Jadaan Riyadh, and ESENTERIC FIBROMATOSIS is a rare benign intraabdominal tumor...

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Mesenteric Fibromatosis: By Saud

Case Report and Literature

A. Al Jadaan

Riyadh,

and

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

REPORT

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

Al Rabeeah

SaudiArabia

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

M

Abdullah

Review

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.

WORDS:

Mesenteric

After appropriate investigations, pathology report confirmed the Mesenteric fibromatosis in chila management challenge for the 132.

Copyright

fibromatosis,

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1999

by

W.B.

intraabdominal

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

1130

Fig 1.

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Abdominal

Surgery,

CT shows

Vol34,

large soft tissue

No 7 (July),

mass.

1999: pp 1130-1132

MESENTERIC

FIBROMATOSIS

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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.

lntraoperative

photo

shows

the

mass

in the

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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ACKNOWLEDGMENT

bowel

The authors

thank

Dr Alex

Gtllis

for reviewing

the manuscrtpt

REFERENCES 1. Enzinger FM, Weiss SW: Fibromatoses. in Enzinger FM, Weiss SW (eds): Soft Tissue Tumors. St Louis, MO, Mosby, 1995, pp 201-229 2 Burke AP, Sobin LH. Shekitka KM, et al: Intra-abdominal fibromatosts: A pathologtc analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Path01 14:335-341, 1990 3. Suarez V, Hall C: Mesenteric fibromatosis. Br J Surg 72:976-978,1985

4. Lotfi AM, Donzois RR, Gordon L: Mesenteric fibromatosis complicating familial adenomatous polyposts: Predrsposmg factors and results of treatment. Int J Colorect Dis 4:30-36, 1989 5. Kyle SM, Keenan RA: Mesentertc fibromatosis preventing restorattve proctectomy. Aust N Z J Surg 62:240-241. 1992 6. Keusch CF. Bauer J: Mesenteric fibromatosis in Gardner’s Syndrome. The Mount Sinai Journal of Medicine 56:318-320, 1989

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7. Suehim S, Inai K: Mesenteric fibromatosis in familial polyposis: Acase report and a review of Japanese cases. Acta Path01 Jpn 37:1837-1843,1987 8. Slater G, Greenstein AJ: Mesenteric fibromatosis in Crohn’s Disease. J Clin Gastroenterol22:147-149, 1996 9. Roger D, Bonnetblanc JM, Leroux-Robert C: Melorheostosis with associated minimal change nephrotic syndrome, mesenteric fibromatosis and capillary haemangiomas. Dermatology 188: 166-168, 1994 10. Yamaguchi K, Hirakata R, Maeda S, et al: Spontaneous isolated intra-abdominal mesenteric fibromatosis. Eur J Surg 157:293-296, 1991 11. David SS, Khanduri P: A case of primary mesenteric fibromatosis. Aust N Z J Surg 62:813-814, 1992 12. Remmele W, Mtiller-Lobeck H, Paulus W: Primary mesenteritis, mesenteric fibrosis and mesenteric fibromatosis, report of four cases, pathology, and classification. Path Res Pratt 184:77-85, 1989

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13. Yannopoulos K, Stout AP: Primary solid tumors of the mesentery. Cancer 16:914-927, 1963 14. Mecrow IK, Miller V, Lendon M, et al: Mesenteric fibromatosis presenting with ascites in childhood. J Pediatr Gastroenterol Nutr 11:118-122,199o 15. Turken A, Senocak ME, Kotiloglu E, et al: Solitary intestinal fibromatosis mimickmg malabsorption syndromes. J Pediatr Surg 30:1387-1389, 1995 16. Al-Salem AH, Al-Hayek R, Qureshi SS: Solitary intestinal fibromatosts: A rare cause of intestinal perforation in neonates. Pediatr Surg Int 12:437-440, 1997 17. Kinn AC, Haggmark T, Willems JS: Aggressive mesenteric fibromatosls, case report. Acta Chir Stand 155:293-296, 1989