Thoracoabdominal duplication accompanied by intestinal malrotation: a case report and literature review of a rare congenital anomaly in an infant.

Introduction and importance: Thoracoabdominal duplication and intestinal malrotation are extremely rare congenital alimentary tract anomalies that can manifest in any segment of the gastrointestinal tract. Still, tubular duplications are an even rarer subset of alimentary tract duplications. Misdiagnosis could occur and this will yield devastating ramifications. Therefore, consideration in the clinical settings is warranted to aid in conducting timely therapeutic interventions. Case presentation: In this article, we illustrate the overwhelmingly rare occurrence of thoracoabdominal duplication coexistent with intestinal malrotation in a 7-month-old male whose primary complaint was chronic dyspnoea since birth that progressed to involve cough and fever. Imaging analysis revealed a significant intrathoracic fluid-filled cyst. Clinical discussion: The intestinal malrotation was treated through Ladd's procedure, and surgical excision of the duplicated segments was accomplished. The subsequent analysis of the resected specimens via means of histopathology utilizing Hematoxylin and Eosin dyes established the definitive diagnosis of a foregut duplication cyst. Conclusion: Thoracoabdominal duplication is one of the most crucial topics in the field of Paediatric Surgery. It is exceptionally rare in occurrence, and the scarcity of available resources that document and describe this topic is evident in the published literature. The authors must opt to document, study, and broaden awareness regarding this life-threatening pathology so that they can circumvent the resultant complications by means of early detection and the performance of apt surgical interventions. Upon careful review of the available literature, we can state that ours is the first-ever case documented from their country regarding this topic and this co-incidence.

Dual unifocal primary Epithelioid Angiosarcoma: A case report and review of the literature unveiling a rare genre of neoplasia.

INTRODUCTION AND IMPORTANCE: Angiosarcomas are an exceedingly rare and malignant form of soft tissue sarcoma that are derived from endothelial cells. Overall, they comprise <1 % of the total number of soft tissue sarcomas. Due to nonspecific and misleading symptoms, the subsequent clinical presentations can easily result in misdiagnosis. This leads to life-threatening complications for patients. Contemplating this tumor as a differential diagnosis during the preoperative phase allows for essential time-sensitive therapeutic interventions to be accomplished. CASE PRESENTATION: Herein, we present the seldom precedented case of a 66-year-old Middle Eastern male who came to our surgical clinic chiefly complaining of an exacerbation of chronic left hypochondriac pain accompanied by gradual inexplicable abdominal distention. Our diagnostic radiological evaluation demonstrated two isolated abdominal mass formations. CLINICAL DISCUSSION: Sheer excision of the neoplastic masses with safety margins was successfully executed via open surgery. The stemming histopathological examination through Hematoxylin and Eosin and immunohistochemical staining established the definitive diagnosis of an Epithelioid Angiosarcoma. CONCLUSION: Epithelioid Angiosarcomas belong to the category of profoundly rare tumors. The available published literature conveys this rarity through the scarcity of epidemiological parameters and studies. It necessitates being borne in mind when facing similar clinical scenarios so that apt therapeutic interventions can be achieved. Structured diagnostic methods, timely surgical interventions and proper techniques, and comprehensive follow-up patient surveillance protocols are, therefore, merited. After thorough review of the published literature, we reckon herewith that ours is the first documented case from our country of an Epithelioid Angiosarcoma.

Unraveling a rare splenic pathology: a case report of a benign hemorrhagic spleen with primary congenital splenic epidermoid cysts.

Introduction and importance: A primary congenital splenic epidermoid cyst is an immensely rare pathology with mostly unknown epidemiological parameters. Misdiagnosis can easily happen and this results in life-threatening ramifications for patients. Considering this pathology as a potential differential diagnosis allows for the required surgical intervention to be timely accomplished. In this case, the authors are documenting this pathology and presenting how it was successfully managed via proper and informed preoperative analysis and meticulous intraoperative technique. Case presentation: Hereby, we portray the exceptionally rare case of a 7-year-old male who presented to our surgical clinic complaining of a sudden left hypochondriac pain with early satiety for 1 month's duration. The preoperative radiological assessment displayed numerous splenic cystic lesions throughout the splenic parenchyma. Clinical discussion: Resection of the cysts was accomplished via total splenectomy. The ensuing histopathological analysis via Hematoxylin and Eosin of the resected specimens established the diagnosis of a congested hemorrhagic spleen with multiple primary congenital splenic epidermoid cysts. Conclusion: Primary congenital splenic epidermoid cysts are an extremely rare type of splenic pathology. There is profound scarcity amidst the published literature regarding it. This merits in-depth study and apt documentation to raise awareness regarding this pathology as a potential differential diagnosis in cases of abdominal pain. Documentation allows us to set up proper and innovative clinical and surgical protocols for these patients. Based on our conclusive review of the published literature, the authors conclude that ours is the first ever documented case from our country of a primary congenital splenic epidermoid cyst.

An enormous benign primary retroperitoneal mucinous cystadenoma: a case report and literature review of a seldom seen abdominal pathology.

Introduction and importance: Retroperitoneal neoplasia comprise less than 2% of all tumours. benign primary retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare tumour. Their clinical course is overall silent unless the patient presents with a vague abdominal or pelvic pain, abdominal distention, or a palpable mass. Their aetiology remains theorized and since 1989, only 46 cases (excluding ours) worldwide were documented in the literature. The majority of cases were discovered in females but the overall tumour incidence rate is still undetermined due to its rarity. Well-timed recognition of this pathology permits the necessary curative surgical intervention to take place. Case presentation: We hereby illustrate the rare case of a 23-year-old female who presented to the surgical clinic complaining solely of an unexplained gradual increase of the abdominal contour. Their presurgical radiological analysis yielded an intraabdominal large-sized well-demarcated retroperitoneal mass. Clinical discussion: Thorough resection of the mass was accomplished via open surgery. The subsequent microscopic analysis of excised tumour yielded the diagnosis of primary retroperitoneal mucinous cystadenoma of benign nature. Conclusion: Primary retroperitoneal mucinous cystadenoma is a seldom seen tumour. The scarcity of its occurrence is further highlighted by the published data. Based on their conclusive review of the available published English-based literature, ours is the 47th documented case of a benign PRMC and it is the first documented case from our country; Syria. The impact of these findings warrants raising awareness on the subject and considering PRMC as a differential diagnosis when presented with a similar case in the clinical practice.

Bouveret Syndrome simultaneous with an incidental solitary Gastric Neuroendocrine Tumor in a 44-year-old female - A Case Report.

Bouveret Syndrome (BS) is a rare type of gallstone ileus, where a gallstone passes via a cholecystoduodenal fistula and causes gastric outlet obstruction. It comprises 0.3-0.5% of cholelithiasis complications. It primarily affects females with an average incidence age of 74 years. Gastric Neuroendocrine Tumors (G-NETs) make up merely 2% of all forms of gastric neoplasia and are known to be an extremely rare occurrence. Their annual incidence is estimated to be one to two per 1 million individuals, and they comprise 8.7% of all known neuroendocrine neoplasia of the gastrointestinal system. Case presentation: We present the case of a 44-year-old Middle Eastern female who presented to the clinic due to multiple episodes of food containing biliary nonprojectile emesis accompanied by epigastric pain. Preoperative radiological workup revealed a Bezoar blocking the gastric outlet and a G-NET in the mucosal layer of the stomach. Clinical discussion: Surgical intervention consisted of excising the impacted calculus to relieve the existing gastric outlet obstruction simultaneous to performing an uncut Roux-en-Y to treat the G-NET condition. The patient underwent complete recovery. Conclusion: BS is an extremely rare form of gallstone ileus and gastric outlet obstruction. Its clinical presentation is nonspecific and results in misdiagnosis. Additionally, it is rare to occur in our patient's age group. NETs are also profoundly rare forms of neoplasia. To the best of our knowledge, no previous cases of simultaneous BS and G-NET have been documented. Therefore, clinical awareness should be raised to timely implement the necessary therapeutic interventions.

Diagnosis and surgical management of a rare neoplastic entity: Solid Pseudopapillary Neoplasm of the Pancreas.

INTRODUCTION AND IMPORTANCE: Solid Pseudopapillary Tumors of the Pancreas is an extremely rare pancreatic neoplastic entity that makes up barely 3 % of all types of exocrine pancreatic neoplasia. Symptoms vary and none of them are specific or pathognomonic for the disease. Therefore, delayed treatment or misdiagnoses could be the result. In turn, patients' morbidity and mortality significantly rise. Diagnosing or suspecting this type of critical type of neoplasia in the preoperative phase is a key component to performing appropriate and curative surgical interventions that result in increased patient survivability. CASE PRESENTATION: We hereby present the rare case of a previously healthy 20-year-old female whose chief complaints were chronic epigastric and left hypochondriac region pain and discomfort along with loss of appetite. During our preoperative radiological investigation, we found a cystic mass with well-defined borders located between the head of the pancreas and the second part of the duodenum. It measured (63 × 45 mm). No metastasis or lymph node involvement was elicited. CLINICAL DISCUSSION: The tumor was utterly resected via a successful Whipple procedure. A definitive diagnosis of a Solid Pseudopapillary Tumor was reached following meticulous histopathological and immunohistochemical analysis of the resected specimens. CONCLUSION: Based on our review of the published literature, no previously published cases from our country of pancreatic Solid Pseudopapillary Tumors exist. Documentation of this rare neoplasia is warranted to raise awareness and to establish the necessary clinical protocols to optimally diagnose, timely treat, and adequately follow up on patients who present with this malignancy.