Cancer being a prothrombotic state, frequently has vascular complications, venous thrombosis, embolism, recurrent venous thromboembolism and a high frequency of anticoagulant failure. We present a rare case of anticoagulant-resistant, progressive, multifocal venous thrombosis and gangrene in all four limbs in a patient with carcinoma gallbladder. A 49 year old lady with locally advanced gallbladder cancer who had been on routine perioperative deep venous thrombosis (DVT) prophylaxis presented two months later with deep venous thrombosis of both lower limbs progressing to venous gangrene of both feet, despite being on anticoagulation. 7 days later, she presented with venous gangrene of both hands. Shortly thereafter, she developed right facial paralysis due to thrombus in the segmental branch of the left MCA despite being on anticoagulation. The hypercoagulable state in cancer involves procoagulant molecules produced by tumor cells, suppression of fibrinolytic activity and platelet activation and is contributed by interactions between the coagulation cascade, complement pathway and immune system. Upto 15% of patients with cancer will develop DVT following surgery, despite standard DVT prophylaxis. Extended DVT prophylaxis should be considered in high-risk patients. Patients with metastases should continue with indefinite anticoagulant therapy after a thrombotic event. In patients without metastasis, anticoagulant treatment is recommended for as long as the cancer is active and while the patient is receiving antitumor therapy. This rare case has been presented to highlight the hypercoagulable state of cancer, the importance of long-term anticoagulation in advanced and metastatic cancers and the high rate of anticoagulation failure associated with unfavourable tumor biology.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma with aggressive local behavior but with a low metastatic potential. Although slow growing and indolent, they rarely reach huge sizes. Very uncommonly, these locally invasive tumors undergo fibrosarcomatous transformation with a more aggressive clinical course, with higher rate of recurrence risk and distant metastases. A 32-years-old lady, presented with a gradually progressive lump in the upper central back for the past 6 years, with rapid progression in size during the last 6 months. On examination, she had a single lump of size 18×18 cm in the midline of the upper back, with prominent veins over its surface. Magnetic resonance imaging (MRI) showed no connection with the spinal canal and appeared flush with the paraspinal muscles. Core needle biopsy showed DFSP. The patient underwent a wide local excision with split skin grafting. The histopathology now showed a fibrosarcomatous transformation of DFSP. The patient again underwent a wide re-excision with a 3 cm margin. Histology reported no evidence of tumor cells in the specimen. The patient’s postoperative period was uneventful and she was referred for adjuvant radiotherapy. DFSP is a rare, slow-growing malignant fibroblastic mesenchymal skin tumor with low metastatic potential. However, in any patient with long standing DFSP with a recent increase in size, this fibrosarcomatous transformation must be kept in mind as it represents an uncommon form of DFSP that tends to follow a more aggressive clinical course, with higher rate of recurrence risk and distant metastases.
Trichobezoar is a rare clinical entity in which a ball of hair accumulates within the alimentary tract. When the tail of the trichobezoar extends into the small intestine, this condition is called Rapunzel syndrome (RS). A 14-year-old female presented with pain abdomen and vomiting for 2 weeks, and a history of trichotillomania and trichophagia, and an epigastric lump. A contrast enhanced computerized tomography (CECT) of the abdomen showed a grossly distended stomach with a heterogeneous mass containing trapped air with underlying normal mucosa suggestive of trichobezoar, with its tail extending into the proximal jejunum suggestive of RS. During laparotomy, a giant trichobezoar was seen in the stomach with its tail extending beyond the duodenum into the proximal jejunum. The entire specimen was delivered out intact. On follow up, she has no surgical complications, and was on behaviour therapy. Trichobezoars form when ingested hair strands become retained in the folds of the gastric mucosa and becomes entangled, forming a ball too large to exit the stomach. Trichotillomania and trichophagia are seen in many of these patients. Patients present with abdominal pain, vomiting, gastric outlet obstruction, and an epigastric mass. In the case of RS, complete removal without breakage and distal migration is important. RS should be considered as a differential diagnosis in a young girl with abdominal pain, vomiting, anaemia and upper abdominal lump. Early diagnosis prevents complications. Surgical removal is treatment of choice. Trichobezoar often coexists with psychiatric illness. Psychiatric evaluation, counselling and treatment are helpful in preventing recurrence.
One of the commonest causes of small intestinal obstruction in India is tuberculosis. The terminal ileum and ileocaecal junction are the most common sites of small bowel involvement, and the most common abnormality is short-segment strictures. We report a patient who presented with small intestinal obstruction due to a solitary ileal stricture, but turned out to be a rare condition that can mimic intestinal tuberculosis. A 60-years-old man, with recurrent episodes of pain abdomen for 1 year, presented to our surgical emergency with evidence of small intestinal obstruction. A CT scan revealed a short segment stricture (2.7 cm) in mid ileal loop with luminal narrowing and marked proximal dilation of proximal ileal loops with a few sub centimetric mesenteric lymph nodes. On laparotomy with a suspected diagnosis of intestinal tuberculosis, it turned to be an adenocarcinoma of the ileum- a rare condition that can mimic intestinal tuberculosis. One of the commonest causes of small intestinal obstruction in India is abdominal tuberculosis. However, all cases of small intestinal obstruction are not tuberculosis. Adenocarcinoma of ileum is a very rare entity. This case report shows that a surgeon should have a high index of suspicion in an elderly patient presenting with small intestinal obstruction, with CT demonstrating isolated short-segment small bowel thickening, as it could be a presentation of obstructing type adenocarcinoma of the small intestine.
Almost all pancreatic neoplasms, including the commonest pancreatic ductal adenocarcinoma (PDAC) are derived from pancreatic epithelial components. Primary pancreatic lymphoma (PPL) is rare, accounting for less than 0.5% of all primary pancreatic tumours and presents a diagnostic and therapeutic challenge owing to their rarity, difficult access, and clinical and imaging features, which can mimic PDAC. A 26-year-old man presented with pain abdomen, jaundice, anorexia and weight loss for two months, along with two episodes of hematemesis and melena. Examination revealed an icteric patient with no palpable abdominal lump. His had a serum bilirubin of 13.6 mg/dl. His ultrasound abdomen, CT scan and MRCP showed a mass in the head and uncinate process of the pancreas with circumferential nodular thickening in second part of duodenum. Side viewing endoscopy was suggestive of carcinoma head of pancreas with duodenal infiltration, for which endoscopic biopsy was taken which revealed non-Hodgkin's lymphoma B cell type. A PET CT showed a metabolically active mass lesion in the head of pancreas, with no other focus of disease anywhere else, suggestive of primary pancreatic lymphoma. This patient was referred to Medical oncology and started on chemotherapy. Lymphoma should be considered when a large, homogeneous, hypoenhancing mass is encountered in the pancreatic head, and with bulky lymphadenopathy. Preoperative tissue diagnosis such as fine needle aspiration cytology with or without flow cytometry and/or laparoscopy/endoscopy guided tissue biopsy may be necessary. It is crucial to differentiate PPL from pancreatic adenocarcinoma since their treatment is primarily medical and prognosis differs considerably.
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