Polycystic liver disease: An uncommon genetic condition.

Key Clinical Message: Timely recognition, accurate diagnosis, and proper management are vital for preventing complications and improving outcomes in polycystic liver disease. Abstract: Polycystic liver disease is an uncommon genetic condition characterized by the presence of over 20 liver cysts. It is symptomatic in only 5% of cases. Surgical intervention remains the primary treatment approach for managing symptoms in affected patients. Herein, we report a case of PLD revealed by severe abdominal pain.

Uncommon Thigh Mass in Neurofibromatosis Type 1: Unveiling Aggressive Epithelioid Sarcoma.

Background: Patients with neurofibromatosis type I (NF1) have an increased risk of developing soft-tissue sarcomas, particularly those related to the nervous system. Epithelioid sarcoma (ES) is an exceptionally rare subtype of soft-tissue sarcoma, with limited knowledge about its clinical presentation and optimal management in NF1. This report aims to provide insights into the characteristics and outcomes of ES in NF1 patients. Case description: A 37-year-old man with a history of NF1 presented with a progressively worsening mass on his right inner thigh. An MRI scan revealed a well-defined tissue mass originating from the adductor magnus muscle, later confirmed as ES through histopathology and immunohistochemistry. Considering poor local and general prognosis, the multidisciplinary team recommended salvage hip disarticulation, however the patient refused and opted for palliative marginal resection to reduce the tumour size. The patient's condition declined rapidly, and he succumbed six days after the surgery. Conclusion: This case highlights the rarity of ES in NF1 patients and underscores the potential for malignant tumour development in this population. Further research is needed to improve our understanding and management of sarcomas in the context of NF1. LEARNING POINTS: Patients with neurofibromatosis type 1 or von Recklinghausen's disease have a higher risk than those with other types of neurofibromatosis of developing benign or malignant soft-tissue tumours especially related to the nervous system.Epithelioid sarcoma is an extremely rare subtype of soft-tissue sarcoma and is exceptionally associated with neurofibromatosis type 1.A multidisciplinary approach remains essential in the diagnosis, management, and treatment of soft-tissue sarcomas in patients with neurofibromatosis type 1.

An unusual cause of inguinal swelling: Primary synovial osteochondromatosis.

Inguinal swelling should raise suspicion for hip joint osteochondromatosis. Early recognition, accurate diagnosis, and prompt surgical intervention are essential for optimizing patient outcomes.

Unveiling the intriguing grape-like mass: Uterine cervical adenosarcoma.

Key Clinical Message: Adenosarcoma of the uterine cervix should be considered in the evaluation of post-menopausal bleeding, as it can be a potential underlying cause. Timely diagnosis and appropriate management are essential to optimize patient outcomes. Abstract: Adenosarcoma is a biphasic neoplasm comprising both a benign epithelial component and a typically low-grade sarcomatous stromal component. Adenosarcoma mainly affects the endometrium (71%), with a lesser incidence in the cervix (2%). Herein, the authors report a case of adenosarcoma of the uterine cervix with distinct gross features.

Rare non-trophoblastic vascular placental tumor: A diagnostic challenge.

Key Clinical Message: Pathologists should consider the differential diagnoses of placental chorioangioma, which encompass chorangiosis, chorangiomatosis, chorangiocarcinoma, intervillous thrombus, and placental infarct. Abstract: Chorioangiomas are rare non-trophoblastic vascular neoplasms of the placenta, occurring in approximately 1% of pregnancies. Large chorioangiomas (>4 cm) are infrequent and associated with an increased risk of pregnancy complications. There are limited reports in the literature regarding uncomplicated cases of large placental chorioangiomas.

Primary giant hydatid cyst of the thigh: An unusual location.

Key Clinical Message: Primary hydatid cyst of the thigh is a rare condition requiring prompt recognition and accurate diagnosis for timely management and prevention of complications. Abstract: Muscular hydatid cysts are rare, with a frequency ranging from 1% to 5% even in endemic areas. The clinical presentation of muscular hydatidosis is typically subtle and lacks specific features, frequently resulting in delayed diagnosis. Herein, the authors describe a case of a primary hydatid cyst located in the thigh.

Synchronous occurrence of hepatic mucinous cystic neoplasm and serous ovarian cystadenoma.

Key clinical message: The coexistence of hepatic mucinous cystic neoplasm and ovarian serous cystadenoma is rare and can only be confirmed by histological examination of the surgical specimen. Complete surgical resection is the optimal treatment. Abstract: Mucinous cystic neoplasms (MCN) of the liver are rare premalignant lesions that account for less than 5% of all hepatic cysts. The synchronous occurrence of hepatic MCN with ovarian cystadenomas is rare. Herein, we report a case of a hepatic MCN coexistent with a serous ovarian cystadenoma.

A rare pancreatic neoplasm in a 40-year-old male patient.

Key Clinical Message: The differential diagnoses of solid pseudopapillary neoplasm of the pancreas include cystic pancreatic neuroendocrine tumor, acinar cell carcinoma, and pancreatoblastoma. Abstract: Solid pseudopapillary neoplasm (SPN) is a low-grade malignant pancreatic tumor which accounts for 0.9%-2.7% of all exocrine pancreatic neoplasms. It predominantly affects young females (90%) and less frequently occurs in male patients. Its prognosis after surgical resection remains excellent. Herein, we report a case of SPN in a male patient.

An uncommon paratubal cyst: Serous borderline tumor.

Paratubal cysts are almost always benign, with rare reports of paratubal serous borderline tumors (PSBT). Only 10 cases of PSBT have been reported in the literature. Their treatment in young patients relies on fertility-preserving surgery. Herein, the authors report a case of PSBT revealed by chronic pelvic pain.

Submucosal micropapillary adenocarcinoma of the sigmoid colon: A distinct and aggressive subtype of adenocarcinoma.

Colorectal invasive micropapillary carcinoma (MPC) is a rare and distinct variant of adenocarcinoma with high incidence of lymph node metastasis. The differential diagnoses of MPC include extensive lymphovascular invasion, metastatic MPC, and retraction artifacts.

Goblet cell adenocarcinoma of the ascending colon: An underrecognized diagnostic pitfall.

Goblet cell adenocarcinomas (GCA) are infrequent neoplasms of the digestive system that exhibit both mucinous and neuroendocrine differentiation. They predominate in the appendix and rarely involve the colon. Herein, the authors report a case of GCA involving the ascending colon in a 60-year-old woman who presented with severe anemia.

Laparoscopic resection of a uterine lipoleiomyoma.

Uterine lipoleiomyomas are rare variants of leiomyomas that usually occur in postmenopausal women. Their incidence ranges from 0.03% to 0.2%. Few cases of laparoscopically resected lipoleiomyomas have been reported in the literature. Further accumulation of lipoleiomyomas could help establish an accurate preoperative diagnosis of this uncommon and benign mesenchymal tumor.

Sarcomatoid carcinoma of the gallbladder: A rare and aggressive form of gallbladder cancer.

Sarcomatoid carcinoma is a rare type of gallbladder cancer with less than 100 cases reported in the literature. Preoperative diagnosis is difficult due to nonspecific clinical and radiological findings. Its prognosis is poor due to rapid progression and a high short-term recurrence rate. The definitive diagnosis relies on histopathological examination.

Pulmonary aspergilloma with prominent oxalate deposition.

Some Aspergillus species produce oxalic acid, which reacts with tissue calcium or blood to precipitate calcium oxalate. Oxalate crystals can induce lung and kidney damage. The presence of oxalate crystals can suggest the diagnosis of aspergillosis, even when Aspergillus hyphae are absent on microscopic slides.

Mucinous breast carcinoma with neuroendocrine differentiation: Case report.

Mucinous carcinoma of the breast is rare representing 2% of all breast carcinomas. It can be classified as either a pure or a mixed type. Pure mucinous breast carcinoma has a less aggressive behavior compared with mixed breast mucinous carcinoma. The latter shows frequent neuroendocrine differentiation.

Tuberculous abdominal cocoon: A rare variant of peritonitis.

Cocoon abdomen is an uncommon condition characterized by bowel entrapment in a cocoon-like membrane. Its clinical presentation is usually non-specific. Rarely, it may present with perforation peritonitis. The therapeutic approach depends on the stage of the disease. Surgery is essentially reserved for those with acute surgical complications.

A rare soft tissue tumor of the big toe: Superficial acral fibromyxoma.

Superficial acral fibromyxoma (SAF) is a rare and benign soft tissue tumor that has a predilection to develop in acral sites. Its treatment is surgical excision. Follow-up is recommended due to the risk of recurrence. The purpose of this case is to raise awareness of this recently described neoplasm.

An unusual simultaneous occurrence of gastric carcinoma with lymphoid stroma, calcified leiomyoma and ectopic pancreas.

Gastric carcinoma with lymphoid stroma is a rare variant of gastric carcinoma accounting for 1%-7% of gastric carcinomas. Its association with gastric leiomyoma and ectopic pancreas is extremely rare. We herein report an unusual simultaneous occurrence of gastric carcinoma with lymphoid stroma, calcified leiomyoma, and ectopic pancreas.

Pseudoxanthomatous salpingitis: An uncommon lesion of the fallopian tube.

Pseudoxanthomatous salpingitis (PXS) is an uncommon condition characterized by the presence of pigment-laden histiocytes within the lamina propria of the fallopian tube. Less than 30 cases of PXS have been reported in the literature. We herein report a case of PXS associated with an endometriotic cyst.

Adenosquamous carcinoma of the pancreas: A rare and aggressive variant of pancreatic cancer.

Adenosquamous carcinoma of the pancreas is a rare and aggressive histological subtype of pancreatic adenocarcinoma, which accounts for 1% to 4% of the exocrine pancreatic malignancies. Its diagnosis relies on histological examination revealing the coexistence of ductal adenocarcinoma and squamous carcinoma with the latter representing ≥30% of the tumor.