Introduction: Pheochromocytomas are rare catecholamine-secreting tumors with a high potential for recurrence post-surgery, necessitating prolonged follow-up. This case highlights the diagnostic and therapeutic challenges in managing recurrent pheochromocytoma. Case presentation: A 25-year-old female, with a history of left pheochromocytoma treated with adrenalectomy a decade earlier, presented with a right adrenal mass. Despite controlled hypertension, elevated urine metanephrines suggested recurrence. Imaging showed a right adrenal mass and suspicious left paraaortic lymph nodes, confirming the diagnosis of recurrent malignant pheochromocytoma in the left adrenal bed and right adrenal gland, with metastasis to the paraaortic lymph nodes. The patient underwent right adrenalectomy coupled with cytoreductive surgery (CRS) in the form of excision of left-sided adrenal bed recurrence and left paraaortic lymph node dissection, intraoperative radiation therapy (IORT), and hyperthermic intraperitoneal chemotherapy (HIPEC). On follow-up six years later, the patient remains free from recurrence. Discussion: This case illustrates the importance of continued surveillance in pheochromocytoma patients, even those with a low-risk profile. The recurrence in this case, despite a smaller initial tumor size and no genetic predispositions, underscores the unpredictable nature of pheochromocytomas. The successful management with CRS, IORT, and HIPEC emphasizes the need for a personalized and multifaceted treatment approach. Conclusion: Pheochromocytoma patients, including those initially considered low risk, require long-term monitoring due to the risk of recurrence. The utilization of CRS, IORT, and HIPEC in this case was pivotal in managing the recurrent and metastatic malignant disease effectively, demonstrating the significance of a comprehensive, multidisciplinary treatment strategy in such complex cases. Highlights:
Gallbladder cancer (GBC) is a rare and highly aggressive malignancy, often characterized by nonspecific clinical presentations and late diagnosis, which contribute to its poor prognosis. It is commonly detected at advanced stages, leading to low survival rates. Surgical resection is the primary treatment, with the extent of surgery depending on the T stage of the cancer. In advanced cases, surgery is only considered if it can potentially be curative. Despite various treatment approaches for advanced GBC, survival outcomes remain poor. In our case series, we introduce a novel treatment approach combining cytoreductive surgery, intraoperative radiation therapy, and hyperthermic intraperitoneal chemotherapy. Remarkably, we observed a 100% one-year survival rate, with one patient achieving eight years of disease-free survival without recurrence or metastasis. This aggressive treatment strategy did not lead to increased morbidity or mortality, suggesting its safety and feasibility. However, larger-scale studies are required to draw definitive conclusions.
Ancient schwannomas are a rare variation of schwannomas, with the distinction being based on histopathological examination of the excised specimen. On histopathological examination, ancient schwannomas exhibit degenerative changes such as calcification, hyalinization, and cystic necrosis, along with S100 positivity. Complete surgical excision is the mainstay treatment for ancient schwannomas and carries a favorable prognosis. Recurrence is the most common complication, often arising from incomplete surgical excision. Herein, we present a case of a 41-year-old male who presented to our center as a case of a retroperitoneal mass for further investigations and diagnostic workup. Imaging showed a retroperitoneal mass in the right iliac fossa. We proceeded with ultrasound guided needle biopsy, and examination of the specimen confirmed the diagnosis of ancient schwannoma. Subsequently, the patient underwent surgery, and complete surgical excision was achieved. On follow-up 3-months later, the patient is doing well, and no signs of recurrence were found.
22 February 2025 in World Journal of Surgical Oncology
The rationale behind the use of HIPEC involves targeted elimination of microscopic peritoneal metastasis, a common route for GCa dissemination, thereby improving the overall survival and reducing recurrences. Moreover, the reasoning behind the use of IORT is enhanced loco-regional control and, therefore, reducing recurrence rates. From February 2013 to June 2023, all GCa patients who underwent HIPEC plus IORT during surgery were included in this study. Median overall survival (OS) and disease-free (DFS) survival were used to evaluate the efficacy of this treatment strategy amongst GCa patients, along with the rate of occurrence and severity of post-operative complications associated with this treatment strategy. The median OS and DFS were 63 and 87 months, respectively. More than one-third of the patients in our cohort did not develop any post-operative complications. In patients who developed post-operative complications, the median number of post-operative complications was 1 (IQR 1–2). Most encountered complications were Clavien-Dindo (CD) grade II complications (33.33%) and no in-hospital mortality was observed. This complex, multimodal treatment strategy results in a significantly prolonged OS and DFS when compared to other treatment strategies for gastric cancer patients, with no added morbidity or mortality.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Less frequently, these tumors can occur outside of the gastrointestinal tract and are termed extragastrointestinal stromal tumors (EGISTs). GISTs and EGISTs share similar molecular and histopathological characteristics; however, the exact pathogenesis of EGISTs is yet to be understood. In this study, we retrospectively reviewed all GIST cases present in the Surveillance, Epidemiology, and End Results (SEER) database. A total of 18 819 patients were included in this analysis, of which 918 had EGIST. Median overall survival and cancer-specific survival (CSS) were not reached in our study. GIST patients had significantly longer CCS than EGIST patients. EGIST, male, regional disease, distant, non-Hispanic Black, age, and radiotherapy were independently associated with shorter CCS in the general population. Contrarily, surgery was a protective factor. Distant disease, increased age, and radiotherapy were independently associated with poor CCS in EGIST patients. In conclusion, surgery should remain the mainstay of treatment in GIST and EGIST patients. The neoplasm appears to be resistant to radiotherapy, and its side effects may worsen patient survival.
Introduction: Jejunal carcinoma, a rare subset of small bowel adenocarcinomas, is characterized by its aggressive nature and nonspecific clinical presentation. The prognosis becomes particularly poor when peritoneal carcinomatosis is present. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has emerged as a treatment performed with curative intent for select patients with gastrointestinal cancers, particularly those with limited peritoneal metastases. Case description: A 51-year-old female presented with progressive symptoms of small bowel obstruction and gastrointestinal bleeding, diagnosed as jejunal adenocarcinoma with limited peritoneal carcinomatosis. She underwent CRS combined with HIPEC. Postoperative recovery was uneventful, with imaging studies and pathological analysis confirming complete tumor resection. Discussion and conclusion: HIPEC and CRS have shown potential in managing peritoneal carcinomatosis. However, their efficacy in rare cancers, such as jejunal carcinoma, remains underexplored. Studies show encouraging outcomes for small bowel adenocarcinoma treated with CRS and HIPEC, emphasizing patient selection and multidisciplinary management.