Júlio Constantino

Undifferentiated embryonal sarcoma of the liver is a rare primary mesenchymal hepatic tumor that usually occurs in pediatric patients. In adulthood, this aggressive neoplasm represents only 7% of the liver sarcomas. This case reports a liver sarcoma occurring in a 49-year-old female patient. The patient was admitted in the emergency room with abdominal pain. Computerized tomography scan and magnetic resonance imaging showed a giant lobulated cystic mass in the right hepatic lobe, suggesting an atypical hemangioma. Right hepatectomy was performed. This rare case promotes a review of the differential diagnosis of liver primary neoplasms including sarcoma. The histological examination revealed an undifferentiated embryonal sarcoma. The patient underwent adjuvant chemotherapy. Currently, our patient is in complete sustained remission 4 years after chemotherapy.

Mechanical intestinal obstruction is a common cause of acute abdominal pain that brings patients to the emergency department. One of the main causes is adhesion in the abdomen after abdominal surgery, but rarer causes exist and are a diagnostic challenge due to the similarity of the presenting symptoms. Here, we present a case of intestinal obstruction caused by diaphragmatic hernia.

Angiomyolipoma (AML) is a rare benign solid tumor, of mesenchymal origin. Angiomyolipomas can be subdivided, according to their predominant component. The predominance of epithelioid cells characterizes the epithelioid variant (EAML). These express a more aggressive clinical behaviour with a greater potential for malignant transformation. In the absence of specific radiological features, the diagnosis of hepatic EAML depends on the pathological and immunohistochemical study. We present the case of an 80 years old female with a computed tomography (CT) scan showing a hepatic nodule at the transition of segments 5 and 8, and a left kidney nodule previously biopsied and confirmed to be a renal angiomyolipoma. A liver nodule biopsy was performed, whose histology revealed a probable gastrointestinal stromal tumor (GIST). The patient underwent a 5/8 subsegmentectomy and pathology report revealed epithelioid angiomyolipoma. After discussion of the case in the multidisciplinary tumor board, it was decided to perform a left nephrectomy, whose pathology revealed leiomyomatous angiomyolipoma. No further treatment and surveillance on outpatient clinic were decided. Hepatic EAML is a rare lesion that, although mostly benign, can exhibit malignant behavior with distant metastasis and local invasion. Its identification is of paramount importance. The definitive diagnosis is only possible through histological and immunohistochemical analysis. Additional studies are needed in order to establish diagnostic criteria and predictive characteristics of malignancy.

The authors present the case of a 51-year-old woman with no history of surgical or traumatic injury or accident, who presented with right hypochondrium and epigastric discomfort, malaise, nausea, loss of appetite and episodes of dark urine and greenish stools. Initial laboratory work-up revealed elevated inflammatory markers including leucocytosis with left shift and C-reactive protein, and a slight elevation of gamma-glutamyltransferase and alkaline phosphatase, with no other significant alterations. Computed tomography (CT) showed intrathoracic acute cholecystitis with a large diaphragmatic hernia. A literature search revealed only one other case of acute cholecystitis complicated by intrathoracic gallbladder due to a non-traumatic diaphragmatic hernia. Symptoms are uncharacteristic and the absence of pain or fever, explained by the altered location of the gallbladder, makes the diagnosis a challenge.

Von Recklinghausen disease (neurofibromatosis type 1—NFT1) is a genetic disorder with autosomal dominant inheritance pattern, caused by mutation of a tumour suppressor gene. Its main features include multiple cutaneous café-au-lait spots and neurofibromas. It is associated with an increased risk of developing neuroendocrine tumours, for instance, in the duodenum. The authors present a case of a 23-year-old male patient admitted to the emergency department due to persistent vomiting. Imaging and biopsy studies revealed an obstructive and large duodenal neuroendocrine tumour; hence the patient underwent a pancreaticoduodenectomy.

Spontaneous posterior rectus sheath hernia is perhaps one of the rarest hernias described only in a few published cases. We present the case of a small bowel herniation through the posterior rectus sheath in a woman without previous abdominal surgical history, resulting in acute small bowel obstruction who was successfully treated by laparoscopy. This is the first case report of this kind of hernia managed with laparoscopic technique. Although the laparoscopic approach is not considered standard treatment for strangulated hernias, in selected cases, it allows the identification and repair of the defect.

Introduction: Iatrogenic vascular injuries during radical nephrectomy are rarely reported. In the case of an injury of the superior mesenteric artery, the consequences for the patient are potentially catastrophic. It occurs more frequently in patients with large renal tumors, due to the presence of bulky perihilar adenopathies, or in cases of pyelonephritis. In most cases, the inadvertent injury of the artery occurs due to the difficulty in distinguishing it from the left renal artery. Case: 69-year-old male, with a malignant neoplasm of the left kidney with the involvement of the tail of the pancreas, tumor thrombus in the left renal vein and multiple left para-aortic adenopathies, whose histological examination revealed to be a renal sarcomatoid carcinoma pT4N1M0G3. A radical nephrectomy was performed with caudal splenopancreatectomy and left paraaortic lymphadenectomy, with an iatrogenic injury of the superior mesenteric artery at its origin. A terminoterminal anastomosis was performed from the proximal stump of the splenic artery. The postoperative period went uneventfully. Control imaging in the follow-up showed permeability of the celiac trunk and the superior mesenteric artery. Conclusions: Intra-operative superior mesenteric artery injury should be promptly identified and repaired to prevent gut ischemia and all its dire consequences. There are different repair options, and the technique should be chosen according to the degree of injury. The splenomesenteric bypass has numerous advantages. Highlights:

Introduction: Boerhaave's syndrome is a life-threatening oesophageal perforation that carries a high mortality rate (20-50%). Diagnosis is difficult by its rarity and the absence of typical symptoms. Treatment of this condition usually requires surgical intervention. Presentation of case: We report the case of a 77-year-old man that resorted to the emergency room with dyspnoea and thoracic pain after vomiting. CT scan revealed pneumomediastinum, left collapse lung and loculated pleural effusion. A left intercostal chest tube was inserted with food drainage. Hence, Boerhaave's syndrome was suspected. Thoracotomy with mediastinum debridement, pleural drainage and oesophageal T-tube drainage was performed. Patient was admitted on the Intensive Care Unit with septic shock, with need for ventilatory support and vasopressor therapy. Two days later, a second look thoracotomy was done with definitive oesophageal repair and pleural patch. The post-operative course was complicated by pneumonia and stroke. Patient was discharged home on the 38th day and remains well at 3 month of follow-up. Discussion: Delayed diagnosis and treatment are the principal causes of high mortality in Boerhaave's syndrome. The classic Mackler's triad (vomiting, lower thoracic pain and subcutaneous emphysema) is present in less then 50% of cases. A thoracic drainage may be useful to confirm diagnosis promptly. There is no standard treatment option. In this case report, the authors used a damage control approach to control sepsis, allowing for a delayed definitive oesophageal repair. Conclusion: Prompt diagnosis with thoracic drainage and a damage control treatment plan might lead to good prognosis for patients with this rare and potentially fatal condition. Highlights: