Heterotopic pancreas, commonly referred to as pancreatic rest or ectopic pancreas, is a congenital anomaly in which pancreatic tissue is anatomically separate from the main pancreatic gland without continuity of a duct system or vascularity. It is commonly found in the upper gastrointestinal tract, specifically in the stomach and small intestine. To date, only about 18 adult cases of heterotopic pancreas in the esophagus have been reported in the English medical literature; seven of which were in women, and five of which originated at the gastroesophageal junction (GEJ). Of these five cases, only two occurred in a hiatal hernia. We report the third case of the heterotopic pancreas at the GEJ in a hiatal hernia discovered in a 62-year-old Caucasian female who presented to the outpatient clinic complaining of worsening reflux characterized as burning retrosternal chest pain. The patient failed maximum medical therapy and was referred to general surgery for laparoscopic hiatal hernia repair with Toupet fundoplication to prevent further reflux and damage to the esophagus. The reflux symptoms persisted after the procedure. Follow-up esophagogastroduodenoscopy with biopsy of the GEJ revealed a small focus of heterotopic pancreas tissue, confirmed by histopathology. The management of heterotopic pancreas differs throughout the literature depending on the size, symptomatology, and potential for malignancy. Management in cases of pancreatic rest, specifically at the GEJ, ranges from observation with conservative medical therapy, resection, or esophagectomy. With this case, we aim to contribute to the literature with the third case of pancreatic rest in the GEJ of a hiatal hernia.
Extramedullary myeloma (EMM) is an infrequent but well-established manifestation of multiple myeloma (MM), defined as a soft tissue plasma cell neoplasm without bone marrow involvement. Gallbladder involvement in EMM, however, is a very rare occurrence, with only 8 cases found in the English medical literature. Here, we present a case of an older adult male with a gallbladder mass in the presence of increasing serum kappa light chains after a normal bone marrow biopsy confirmed the complete remission of a previous MM diagnosis. Histopathologic evaluation of a biopsied sample confirmed the mass as an atypical plasma cell neoplasm. Later in his treatment, he developed several firm, smooth, violaceous skin nodules on the torso, which histopathology confirmed as also being atypical plasma cell neoplasms. We aim to contribute to the medical literature by expanding the pool of information regarding EMM of the gallbladder to support future diagnostic and treatment recommendations.
Langerhans cell histiocytosis (LCH) is a rare disorder involving the proliferation of myeloid-derived dendritic cells. It most commonly affects children aged less than 1 to 2 years old. Langerhans cell histiocytosis in adults is more uncommon with an estimated incidence of 1 to 2 cases per 1 million. Langerhans cell histiocytosis can present as a multisystem or single-system disease involving bone, skin, lymph nodes, and various other organ systems. The spectrum of symptoms can range from asymptomatic disease, localized skeletal or dermatologic manifestations, or systemic symptoms of weight loss, fever, and other organ-specific manifestations. Langerhans cell histiocytosis with isolated involvement of the gastrointestinal tract is exceedingly rare with only approximately 14 cases reported in the English medical literature. Here, we report an additional case of LCH presenting as an isolated colonic polyp. This patient was also followed for a 3-year period after initial diagnosis to provide valuable follow-up data. With this case, we aim to contribute to the literature by further characterizing the presentation, treatment, and disease course of this rare phenomenon and provide valuable data to guide future screening guidelines for isolated LCH polyps in the colon.
Introduction: Composite lymphoma is defined as coexistence of two or more morphologically and phenotypically distinct lymphomas in the same anatomical site. Composite lymphoma may include combinations of Hodgkin lymphoma (HL) and B- or T-cell non-Hodgkin lymphoma (NHL); B-cell NHL and T-cell NHL; or two distinct B-cell or T-cell NHLs. The exact pathogenesis of composite lymphoma is unknown. Most cases demonstrate poor outcomes with a median survival of 12 months. The treatment is usually directed toward the higher-grade component.
Case Report: Here, we report an extraordinarily rare case of a composite lymphoma composed of peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) and follicular B-cell lymphoma (FBCL) coexisting in a single axillary lymph node in a 66-year-old female.
Conclusion: The medical literature lacks significant information regarding this type of composite lymphoma, thus creating a challenge for management. Currently, only one other case of this type of composite lymphoma has been reported in the English medical literature, with this case reporting the first female patient.
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