Malignant melanocytic neoplasm of pancreas with liver metastasis: Is it malignant melanoma or clear cell sarcoma?

Malignant melanocytic neoplasm, usually seen in soft tissues, is rare in a visceral location and presents as a diagnostic dilemma. We present a case of pancreatic malignant melanocytic neoplasm with liver metastasis. A 58-year-old man presented with left upper abdominal swelling and loss of appetite. Imaging revealed a large mass arising from the pancreatic tail, and this was diagnosed as malignant neoplasm with melanocytic differentiation on biopsy with the possible differentials of malignant melanoma, clear cell sarcoma (CCS), and perivascular epithelioid cell neoplasm. The patient underwent distal pancreatectomy and splenectomy for the same. Follow-up imaging 6 months later showed a metastatic liver lesion, for which he also underwent a liver resection. BRAF mutational analysis was found to be negative. Both CCS and malignant melanoma have similar morphological features and melanocytic differentiation, but each harbors a distinct genetic background. Differentiation of both has diagnostic and therapeutic implications.

Blue rubber bleb nevus syndrome: a rare cause of chronic gastrointestinal bleed in adults.

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by venous malformations in the skin, gastrointestinal tract and other parts of the body. Its presentation is usually sporadic, although cases of autosomal dominant inheritance have been reported. Usually seen in children, BRBNS presentation in adults is rare. Symptoms at presentation depend on the organs involved; patients with BRBNS may present with acute or chronic gastrointestinal bleed. We herein report a rare presentation of BRBNS in an adult who suffered from intermittent abdominal pain and melaena for three years. Contrast-enhanced computed tomography revealed a jejunojejunal intussusception with a vascular malformation as the lead point. The patient underwent laparotomy with resection of the intussuscepted bowel segment. Recovery was uneventful. In spite of a wide range of therapeutic options for the management of BRBNS described in the literature, the efficacy of those available therapies, including surgical excision, is not well established.

A Hairy Tail not a Fairy Tale - Rapunzel Syndrome.

Rapunzel syndrome is an unusual and rare type of trichobezoar. Bezoars can be classified according to the primary constituent, as trichobezoar (hair), phytobezoar (plant material) or miscellaneous (pharmacobezoar, lactobezoar, fungal agglomeration and foreign bodies). When a long tail of hair strands extends from the main mass in the stomach along the small intestine and beyond it is known as Rapunzel syndrome. Here we are reporting a case of Rapunzel syndrome with a very long tail who was managed successfully. And reviewing the literature on the pathophysiology and management of these patients. These patients commonly present with obstructive symptoms and needs a high index of suspicion especially, in young female patients who have alopecia circumscripta and underlying psychiatric disorders. Early diagnosis and treatment is required to prevent complications due to this condition. Currently surgical management of this condition is the treatment of choice.

Extended multiorgan resection in locally advanced gastric cancer: a single centre experience from south India.

BACKGROUND: The prognostic and survival benefit of extended multiorgan resection for locally advanced gastric adenocarcinoma remains controversial. The morbidity associated with additional organ resection has been found to be higher when compared to patients undergoing gastrectomy alone. The aim of our study was to evaluate the morbidity, mortality and survival benefit associated with extended multiorgan resection for locally advanced gastric adenocarcinoma. METHODS: From January 2004 to December 2011, 721 patients underwent resectional surgery for gastric adenocarcinoma at the Christian Medical College, Vellore, India. Out of this group, 36 patients underwent primary resection and had one or more organs resected in addition to the stomach. A retrospective analysis of the case records of all these patients was performed. The Kaplan-Meier survival probability was estimated. Cox regression analysis was used to evaluate the clinico-pathological variables affecting the survival of these patients. RESULTS: The perioperative morbidity and mortality rates were 25% and 5.5% respectively. The most common organs resected were colon and spleen. The incidence of pathologically confirmed T4b cancers was only 50%. The median survival of these patients was 28 months. The survival was influenced by a R0 or curative resection. However, it was not statistically significant. CONCLUSION: Extended multiorganresection in locally advanced gastric cancer can be performed with acceptable morbidity and mortality. In our study, overstaging was found in 50% of the patients and hence, when the real nature of invasion is unclear, the surgeon may proceed with en bloc resection of the stomach with the involved adjacent organs. As long as an R0 resection can be achieved, extended multiorgan resection can be performed for carcinoma stomach.

Tracheal injury during oesophagectomy--incidence, treatment and outcome.

BACKGROUND AND AIM: Iatrogenic tracheal injuries are uncommon, potentially lethal and associated with significant morbidity. In this report we analyze the incidence of iatrogenic tracheobronchial injuries sustained during oesophagectomies and the results and outcome of repair using a pedicled intercostal muscle flap. METHODS: A retrospective analysis was done on all patients who underwent an oesophagectomy between June 2000 and May 2011. Data was collected from an electronic database and the medical records of patients, maintained at our hospital. RESULTS: One hundred and fourteen patient records were analyzed. There were 85 male and 29 female patients. Their mean age was 47 years (range 16 to 86 years). Forty two (36%) underwent a transhiatal oesophagectomy, 34 (31%) Mckeown's oesophagectomy, 35 (31%) Ivor Lewis oesophagectomy and 3 (2%) thoracoscopy assisted oesophagectomy. Of the 114 oesophagectomies, 86 (75%) were performed for malignant and 28 (25%) for benign pathologies (benign tumors and corrosive strictures of the esophagus). In our study, four patients sustained injury to the tracheobronchial tree during oesophagectomy. In patients who sustained injury during a transhiatal dissection a right anterolateral thoracotomy was made. All injuries in the thoracic tracheobronchial tree were repaired primarily and reinforced with an intercostal muscle flap. In the patient with injury to the cervical part of the trachea, repair was done primarily and reinforced with cyanoacrylate glue. All patients who sustained injury had malignancy and three patients had received neoadjuvant chemoradiotherapy. Two patients sustained injury during transhiatal oesophagectomy and two during a Mckeown's oesophagectomy. There was one mortality which was due to ventilator associated pneumonia and related complications. The remaining three were alive with no evidence of repair breakdown. CONCLUSIONS: Iatrogenic tracheal injuries are uncommon complications but associated with significant morbidity. Preoperative chemoradiotherapy and malignancy are risk factors for iatrogenic tracheal injuries. Reinforcement of the suture line with a muscle flap is an effective technique of repair. Prompt ontable identification and adequate surgical treatment is necessary for a good outcome.