Multiple Primary Dedifferentiated Liposarcoma of the Jejunal Mesentery: A Case Report and Review of Literature.

Liposarcoma arising primarily from the intestinal mesentery is a rare malignancy. Malignancy is said to be synchronous when there is occurrence of two or more tumours that have not spread from a common site or recurred and show no evidence of metastasis. Multiple synchronous primary liposarcoma of the mesentery is a very unusual clinical finding. Here, we report a rare case of synchronous multiple primary dedifferentiated liposarcoma of jejunal mesentery in a 36-year-old female patient. Radiological investigations aided in making a provisional diagnosis of an ovarian malignancy. A staging laparotomy was performed and general surgeon's help was sought due to the presence of three separate jejunal mesenteric masses of sizes 8x6 cms, 6x6 cms and 25x20 cms respectively. Complete excision of mesenteric masses with one feet of involved jejunum was done and a jejuno-jejunal anastomosis made. The histopathology report was indicative of multiple dedifferentiated liposarcoma of jejunal mesentery. Postoperatively patient received Doxorubicin, Dacarbazine and Ifosfamide based adjuvant chemotherapy in view of poorly differentiated tumour. Patient remains tumour free for the last 12 months of follow up.

Malignant Peripheral Nerve Sheath Tumour of Small Intestine Presenting as Ileo-Ileal Intussusception - A Rare Tumour with Unusual Complication.

Malignant Peripheral Nerve Sheath Tumours (MPNST) arises from a peripheral nerve or exhibit nerve sheath differentiation on histology. Proximal portions of the upper and lower extremities and the trunk are the most common sites of occurrence. Around 50% are associated with Neurofibromatosis Type 1 (NF1) with incidence of two to five per cent in patients with NF1. The estimated incidence in general population without NF1 is 0.0001% of which gastrointestinal MPNST are extremely rare. A 45-year-old lady without pathological antecedent for NF1 was admitted with pain in right lower abdomen and multiple episodes of vomiting for 3 months. Preoperatively intussusception was diagnosed in the small bowel with USG and CECT abdomen showing characteristic target sign. On laparotomy Ileo-ileal intussusception (proximal ileum telescoping into distal ileum) was found 2 feet proximal to ileo-caecal junction with surrounding inflammed mesentery and presence of intraluminal tumour as lead point. Resection of involved segment of ileum along with its mesentery was done followed by ileo-ileal anastomosis. Histopathology was suggestive of high grade MPNST. Postoperative course and follow up for last 10 month is uneventful. This case is unique in terms of a rare tumour presenting with unusual complication and only one case had been reported so far in western literature.

Sialolipoma of the parotid gland: a rare entity.

Lipomatous neoplasms of the parotid gland are rare tumours contributing from 0.6% to 4.4% to all parotid neoplasms with most series reporting an incidence of 1%. It is extremely rare in females, being 10 times more common in males, and the most common age of presentation is the five to sixth decade. Numerous histological variants of salivary gland lipoma have been described. Sialolipoma is a rare variant having proliferation of mature adipocytes with secondary entrapment of the normal salivary gland element. We report a rare case of sialolipoma in the right parotid of a 45-year-old woman who was treated with superficial parotidectomy. Postoperative recovery as well as follow-up has been uneventful.

Non-functional paraganglioma of retroperitoneum mimicking pancreatic mass with concurrent urinary bladder paraganglioma: an extremely rare entity.

Paragangliomas are extra-adrenal tumours of the autonomic nervous system, which rarely present as primary retroperitoneal mass mimicking pancreatic malignancy (incidence 2-8 per million populations). Urinary Bladder Paraganglioma are also extremely rare (0.06% of all Bladder Tumour and 6% of Paragangliomas) with most being malignant and high grade tumours. Non-functional varieties of both tumours are usually incidentally diagnosed. The possibility for malignant transformation in them makes surgical excision the treatment of choice. A 45-year-old lady with abdominal pain was investigated to have a complex retroperitoneal mass behind head of pancreas and a urinary bladder mass. Complete excision of retroperitoneal and bladder lesion was done. Histopathological examinations of both specimens were suggestive of Paraganglioma with no abnormal mitotic activity and capsular/vascular invasion. Although concurrent non functional paragangliomas had been reported but the synchronous non-functional paragangliomas of retroperitoneum and urinary bladder reported in this case is extremely rare and is not reported so far in English literature.