Pneumatosis cystoides intestinalis revealed after a hand-to-hand aggression: A case report.

INTRODUCTION: Pneumatosis cystoides intestinalis (PCI) is a condition defined by the presence of multiple gas-filled cysts within the intestinal wall. We demonstrated a case of PCI presenting as pneumoperitoneum following a hand-to-hand aggression. Consent was obtained from the patient for publication of this paper. PRESENTATION OF THE CASE: This article describes a case of an 28 year-old man with medical history of gastroduodenal ulcer admitted in the emergency room with an acute abdominal pain secondary to a hand-to-hand aggression. Computed tomography (CT-scan) revealed signs of PCI, the presence of pneumoperitoneum and a small amount of fluid in the Douglas pouch. The patient underwent an urgent laparotomy in front of a high suspicion of a bowel perforation. Per operative findings revealed multiple small cysts of the terminal ileum and there were no bowel perforation. DISCUSSION: Pneumatosis cystoides intestinalis may be related to a wide spectrum of gastrointestinal conditions. The diagnosis of PCI can be established by endoscopic ultrasound or CT-scan imaging. Management of PCI is conditioned by the clinical and radiological presentation which is essentially related to the primary cause. Conservative approach is allowed in a stable patient with no signs of complications. In the presence of predictive factors of pathologic PCI, namely transmural ischemia and bowel perforation, surgical operation is required. CONCLUSION: The management of PCI may be challenging particularly in the presence of pneumoperitoneum. Complications must be excluded before considering a conservative therapy. Therefore, PCI should be interpreted with relevance to the entire clinical context.

Metachronous isolated locally advanced pancreatic metastasis from chromophobe renal cell carcinoma.

INTRODUCTION: Metastasis to the pancreas is relatively uncommon occurrence. Isolated pancreatic metastasis from renal cell carcinoma (RCC) is relatively rare and it's usually seen in clear cell renal cell carcinoma (CCRCC), but its occurrence from chromophobe renal cell carcinoma (chRCC) is extremely rare, due to its relatively low-grade metastatic potential. PRESENTATION OF CASE: The authors report an unusual case of metachronous isolated pancreatic metastasis, in a 65-year-old female patient two years after left nephrectomy for chRCC, diagnosed during routine surveillance imaging and confirmed after a CT-guided pancreatic biopsy. DISCUSSION: The pancreas is an elective site for metastases from RCC, and this particularity has been reported by several studies, but only described for CCRCC. In the English literature there is only one case of pancreatic metastasis from chRCC diagnosed in an autopsy study. Surgical resection of metastasis remains the most effective treatment, particularly for pancreatic metastases from chRCC, since radiotherapy, chemotherapy, hormonal therapy, and targeted therapy have generally proved ineffective for metastatic chRCC. In case of an unresectable disease, surgical or endoscopic palliation in association with palliative chemotherapy can improve the quality of life but not survival. CONCLUSION: This case highlights the unique behavior of chRCC with an unusual site of metastasis, and the necessity of long-term follow-up after primary tumor removal, even if it is known for a low-grade metastatic potential and a relatively good prognosis.

Malignant rhabdoid tumor in a solitary kidney arising in an adult patient with chronic obstructive renal calculi.

INTRODUCTION: Malignant rhabdoid tumor (MRT) is one of the most aggressive and lethal malignancies in pediatric oncology, MRT of the kidney is an uncommon renal tumor in children and it's extremely rare in adult patients. With only seven previously reported adult cases in the English-literature, to our knowledge this is the first case that is associated with renal calculi. PRESENTATION OF CASE: We present the case of a 65-year-old man with an MRT arising in a solitary kidney with multiple enlarged lymph nodes that compressing the inferior vena cava. DISCUSSION: Malignant rhabdoid tumor of the kidney was originally described as a "rhabdomyosarcomatoid" variant of Wilm's tumor due to the resemblance of cells to rhabdomyoblasts, now this type of tumor is recognized as distant and unique malignant renal tumor. It affects usually children before the age of 2 years. Tumor tissue sampling is required to make the diagnosis of MRTK, based on either nephrectomy, core biopsy, or autopsy specimens. There is no established standard of care due to the paucity of cases. Surgery is considered to be the first choice of treatment if possible. CONCLUSION: This case report reinforces the importance of recognizing this entity in the adult population, and discuss the possible treatment options of this rare and highly aggressive tumor.

Round cell sarcoma of the colon with CIC rearrangement.

BACKGROUND: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional. CASE PRESENTATION: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression. CONCLUSIONS: The CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.

Adult intussusceptions: Clinical presentation, diagnosis and therapeutic management.

BACKGROUND: Adult intussusception is a rare clinical entity. It is an uncommon cause of intestinal obstruction in adult. It often presents with nonspecific symptoms and preoperative diagnosis remains difficult. The purpose of this study was to determine the clinical entity and surgical approach of adult intussusception. METHODS: We have conducted a retrospective descriptive study starting from 2006 until 2014. We reviewed data for all patients that had been admitted to our department for intestinal intussusception. RESULTS: Eight consecutive patients were admitted to our department. The mean age was 48 years old (20-71). The sex ratio was 0,6. The clinical presentation was acute in 5 cases. A computed tomography was performed in 6 cases. The diagnosis of gastrointestinal intussusception was made preoperatively in 100% of patients. All patients underwent surgery. An organic lesion was identified in 100% of the cases. In all cases, resection of the intussuscepted intestinal loop was done without intestinal reduction. All patients were well followed up and recurrences have been documented. CONCLUSION: In adults, intussusception is usually secondary to an organic cause. In the absence of signs of severity, etiologic diagnosis based on CT allows the diagnosis of the intussusception and sometimes can detect the causal lesion. Therapeutic sanction of intussusception is surgery and there is more emphasis towards resection without reduction.