Loco-regional recurrence of adrenocortical carcinoma: A case report.

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a high recurrence rate. Approximately half of the patients are asymptomatic, while others experience symptoms due to the tumor's size or hormone secretion. Ro resection if possible is the best option for treatment of primary as well as locoregional recurrent ACC. CASE PRESENTATION: A 20-year-old female who previously underwent open left adrenalectomy for Stage III ACC presented with complaints of heaviness and vague discomfort in the left upper abdomen. Current hormonal assays were normal. Imaging revealed a lesion in the spleen suggestive of recurrence. She underwent elective surgery involving en bloc resection of the spleen, diaphragm, and associated structures. Postoperative recovery was uneventful, histopathology confirmed recurrence and subsequent PET-CT showed no recurrence. She is currently on mitotane and remains symptom-free with no signs of recurrence after initial surgery. CLINICAL DISCUSSION: Complete resection (Ro) if possible, for recurrent and metastatic disease has been linked to long-term survival and offers significant palliative benefits, particularly in cases involving symptomatic steroid production. CONCLUSION: ACC has a high frequency of local recurrence therefore management of recurrence should be considered from the initial diagnosis. Ro resection of recurrence is the best potential treatment. Follow-up protocols and improving integration between surgical, oncological, and supportive care departments are crucial for overcoming healthcare challenges in Nepal.

Post-operative Pancreatitis as a Predictor of Postoperative Pancreatic Fistula in Patients Following Pancreaticoduodenectomy.

BACKGROUND: Postoperative pancreatic fistula remains the single most important determinant of morbidity and mortality following pancreaticoduodenectomy. A new entity was proposed by Saxon Connor "Post-Operative pancreatitis", which is defined by raised serum amylase more than the upper limit of institutional serum amylase value on Post-Operative day 0 or 1. There has been shown to be an association between postoperative pancreatitis and postoperative pancreatic fistula. We have conducted this study to see the incidence of postoperative pancreatitis and its association with postoperative pancreatic fistula. METHODS: This was a prospective observational study. All patients undergoing pancreaticoduodenectomy at a tertiary care center for one and a half years were included. A cut-off value of serum amylase 80U/L was used to make a diagnosis of postoperative pancreatitis. The patients were followed up for one month. Pancreas specific complications were defined according to the definition given by the International Study Group of Pancreatic Surgery. RESULTS: A total of 49 pancreaticoduodenectomies were done in the given period. The incidence of postoperative pancreatitis was 31(63.3%) and postoperative pancreatic fistula was 19(38.8%). Postoperative pancreatic fistula was seen in 19(61.2%) of patients having postoperative pancreatitis (P<0.001). Post-operative pancreatitis was also significantly associated with post pancreatectomy hemorrhage, increased hospital stay, and mortality. In multivariate analysis, preoperative endoscopic biliary drainage and increased serum amylase on the first postoperative day came out to be an independent predictor of postoperative pancreatic fistula. CONCLUSIONS: Post-operative Pancreatitis was associated with an increased incidence of Post-operative pancreatic fistula and other postoperative complications like Post pancreatectomy hemorrhage and mortality.

Multiple jejunal gastrointestinal stromal tumors and Neurofibromatosis type 1: A rare association.

INTRODUCTION AND IMPORTANCE: The association between gastrointestinal stromal tumor (GIST), mesenchymal tumor arising from the interstitial cells of cajal and Neurofibromatosis type 1 (NF1), an autosomal dominant disease has been reported in the literature. GIST in NF1 patients are multiple and located in the small intestine. Tumorigenesis in NF1 associated GIST is different to that of sporadic GIST and hence the treatment. Here we report a rare case of an NF1 patient with multiple jejunal GISTs. CASE PRESENTATION: We here present a rare case of a 57-year-old male diagnosed with NF1 30 years back, presented in our emergency department with complaints of black, tarry stools later diagnosed to have multiple GIST in jejunum. Contrast enhanced computed tomography (CECT) of the abdomen showed a large 10.1 × 7.33 × 6.2 cm heterogeneous, exophytic, solid mass with cystic areas originating from the jejunum. The microscopic examination of the specimen showed spindle shaped tumor cells while immunohistochemistry showed CD117 (c-KIT) and DOG-1 positivity. The primary treatment was complete surgical excision of the tumor. CLINICAL DISCUSSION: The incidence of GISTs in NF1 patient is around 6-7%; however, concomitant presence of multiple GISTs is rare. CECT of abdomen along with histopathological and immunohistochemistry studies are diagnostic. The management of GIST includes surgical and adjuvant therapy methods based on the tumorigenesis and recurrent risk stratification. CONCLUSION: Early clinical suspicion and imaging aids in early detection of the tumor in patients with NF1 presenting with gastrointestinal symptoms. Postoperatively, screening for recurrence with radiology is of utmost importance.

Squamous cell carcinoma arising within choledochal cyst managed by pancreaticoduodenectomy: A case report.

INTRODUCTION AND IMPORTANCE: Choledochal cysts (CC) are rare cystic dilatation of the biliary trees. Malignancy is one of the important significant findings in CC and its incidence increases with age. Associated squamous cell carcinoma of the choledochal cyst is an uncommon pathological finding. CASE PRESENTATION: We present a 21-year-old male diagnosed with type 1 CC and planned for excision of the cystic dilated extrahepatic biliary tract with hepaticojejunal anastomosis in Roux-en-Y but underwent pancreaticoduodenectomy due to intraoperative palpable mass of CC which was adhered to the duodenum and pancreatic head. Histopathology of the excised specimen revealed squamous cell carcinoma (SCC) of the choledochal cyst. DISCUSSION: CC represents a rare biliary cystic disease. Though infrequent, malignant transformation in CC includes cholangiocarcinoma, adenocarcinoma, and rarely SCC. The post-operative management for SCC in CC is ill-defined and carries a grave prognosis. CONCLUSION: The choledochal cyst should be completely excised wherever possible to avoid the possible malignant transformation in CC including SCC.