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Indian Journal of Cancer, Vol. 47, No. 2, April-June, 2010, pp. 233-234 Letter To Editor Malignant nerve sheath tumor of the mesentery Goyal V, Thomas S, Pathania OP, Agarwal S Department of Surgery, Lady Hardinge Medical College, Bhagat Singh Road, New Delhi-110 001, India Correspondence Address: Dr. Vinay Goyal, Department of Surgery, Lady Hardinge Medical College, Bhagat Singh Road, New Delhi-110 001, India docvinay2002@yahoo.co.uk Code Number: cn10061 PMID: 20448400 DOI: 10.4103/0019-509X.63021 Sir, We are reporting a rare case of malignant peripheral nerve sheath tumor (MPNST) arising from the small bowel mesentery. There is only one similar case reported in the literature till date. Our patient was a 36-year-old female without any signs of neurofibromatosis, who presented to us with recurrent lump in the abdomen for the past 3 years. In the past 3 years, the patient had undergone excision of lump twice at an outside hospital but each time the lump recurred rapidly within a few months of the surgery. No records or histopathology reports from the previous surgeries were available with the patient. On presentation, the patient had massive lump abdomen [Figure - 1]. The abdomen was grossly distended with multiple scar marks from previous surgeries. There was a huge intraabdominal lump occupying the entire abdomen, extending from under the costal margin to the pelvis. A contrast-enhanced computerized tomography scan showed a large multilobulated heterogeneous intraperitoneal mass suggestive of mesenchymal tumor, extending from the epigastrium to the pelvis. There was no evidence of metastasis. The patient was subjected to a laparotomy with a generous midline incision. A 50 cm x 25 cm x 20 cm tumor was seen in the mesentery of the jejunum with a single loop of jejunum stretched across it. The tumor was extending from the undersurface of the liver into the pelvis. There was no evidence of metastasis. The tumor was excised along with the adherent loop of the jejunum and jejunojejunostomy was performed. Weight of the tumor was 14.6 kg. Histopathology report showed high-grade MPNST of the mesentery. S-100 staining was positive. All the six mesenteric lymph nodes excised along with the tumor showed no evidence of metastasis [Figure - 2]. Postoperative course of the patient was uneventful. MPNSTs are rare soft-tissue tumors accounting for 5-10% of all soft tissue tumors, and up to 50% of these tumors are found in patients with neurofibromatosis type 1 (NF1). [1] The extremities are the most common sites of involvement by these tumors. [1] The estimated incidence of MPNST in NF1 patients ranges between 2 and 5% as compared to 0.0001% in the general population. [1] Most MPNSTs arise in relation with major nerve trunks, like sciatic nerve, brachial plexus and sacral plexus, and the most common sites of occurrence are proximal portions of the upper and lower extremities and the trunk. [1] Mesentery of the small bowel as seen in the present case is a rare site of origin of MPNST, with only four cases reported till date in the literature. [2],[3],[4],[5] Of these, only one case was not associated with NF. [2] Clinically, MPNSTs are high-grade sarcomas with a high rate of recurrences and distant metastasis, with an overall 5-year survival rate of around 52%. [1] Poor prognostic factors include size ≥5 cm, incomplete resection and association with NF1. [1] Our patient did not have NF1 but had tumor size ≥5 cm, which was resected completely. Although previous records were not available, we strongly suspect that this was a third-time recurrence due to incomplete resection previously. The patient underwent radiotherapy of the whole abdomen. No recurrence of the tumor was found on 1-year follow-up. References
Copyright 2010 - Indian Journal of Cancer The following images related to this document are available:Photo images[cn10061f2.jpg] [cn10061f1.jpg] |
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