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[This corrects the article DOI: 10.7759/cureus.37663.].
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Adult T-cell leukemia/lymphoma (ATLL) is a rare form of T-cell lymphoma with poor median survival time and limited response to chemotherapy. We present a 45-year-old female from Ghana with generalized body rash, hypercalcemia, lymphadenopathy, and lytic bone lesions. She had a history of strongyloidiasis, treated two years ago, and her serology was positive for the human T-cell lymphotropic virus type 1 (HTLV-1). Histopathology of cervical lymph node and abdominal rash biopsy revealed T-cell lymphoma. We present a literature review on the topic and the challenges of diagnosis. We emphasize the importance of considering HTLV-1-associated ATLL in patients who have been treated for strongyloidiasis in the past and are presenting with rash or lymphadenopathy.
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IgG4-related disease (IgG4-RD) is an immune-mediated disorder that involves multiple organs and is characterized by the infiltration of lymphoplasmacytic cells, including IgG4-positive plasma cells, along with storiform fibrosis and obliterative phlebitis in the inflamed organs. The primary sites affected by this condition include the pancreas, bile ducts, salivary glands, aorta, lungs, kidneys, meninges, lacrimal glands, mediastinal lymph nodes, and retroperitoneum. The pathogenesis is linked to a type 2 T-helper-cell cytokine profile and the involvement of regulatory T cells. However, the exact mechanism is still unknown. Patients with IgG4-related disease are frequently misdiagnosed as having malignancies due to the resemblance of the lesions to infections or other immune-mediated diseases and certain tumors, such as pancreatic cancer and pseudo-renal pelvis tumor. Prompt identification of IgG4-related disease is essential as a delayed diagnosis until advanced stages can result in severe organ damage and potentially fatal outcomes, despite the disease being highly responsive to treatment. This report presents a highly unusual case of IgG4-related disease (IgG4-RD) with an atypical presentation in a 38-year-old female patient. The patient sought medical attention in the emergency department due to nasal septal erosions and an oral-antral fistula. Nasal cultures were conducted and indicated the presence of Klebsiella ozaena. Subsequent investigations, including a nasal biopsy, confirmed the diagnosis of IgG4-related autoimmune disease.
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Stauffer's syndrome is a paraneoplastic syndrome that has historically been associated with renal cell carcinoma. It is defined by the anicteric elevation of liver enzymes in the absence of liver metastasis, and the reversibility of clinical and biochemical changes upon treatment of the primary pathology. Here, we discuss the rare presentation of Stauffer's syndrome in a patient with advanced metastatic prostate cancer. A 72-year-old male presented with generalized weakness, dizziness, weight loss, and icterus who was incidentally found to have a prostatic enlargement on physical examination. The laboratory investigations and radiographic imaging confirmed the diagnosis of metastatic prostatic cancer without any evidence of mechanical biliary obstruction as confirmed by biopsy and imaging. The cancer had metastasized to pelvic sidewalls, pelvic bones, ribs, urinary bladder, and local lymph nodes. Our case signifies that a high index of suspicion for underlying cancer should be maintained in patients presenting with cholestatic liver dysfunction, with or without jaundice, especially in the absence of a recognizable mechanical etiology of cholestasis.
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Colorectal lymphomas are very rare and are usually found in male patients between the fifth and seventh decade of life. Our patient was a 30-year-old male who presented with the complaint of epigastric pain and abdominal mass for the past three months. Physical examination was remarkable for soft solid abdominal mass extending from the right side of the abdomen toward the left side. Computed tomography showed a large lobulated mass in the right lower quadrant involving multiple loops of bowel. The mass measured 15.1 x 12.5 cm in transverse dimension and 16.2 cm in craniocaudal dimension. Colonoscopy showed a villous and fungating mass occupying the whole cecum and extending into the ascending colon. Pathology from the colonoscopy mass revealed diffuse large B-cell lymphoma, which was CD20 (cluster of differentiation 20) positive. He was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy regimen and is following at the oncology outpatient department. A combination of surgical resection and chemotherapy is used in treating patients with colorectal lymphoma.
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Amyloidosis is a systemic disease that results from the extracellular deposition of an abnormal protein called amyloid. The kidney and the heart are the most common organ affected by amyloidosis while in some cases liver involvement can be seen. Our patient is a 60-year-old African American male who presented to the emergency department because of multiple episodes of syncope over the past day. Chest x-ray and ultrasound chest were suggestive of pleural effusion for which thoracentesis was done. His hospital course was complicated with renal and liver failure. Computed tomography (CT) abdomen and pelvis was done which showed mild hepatomegaly. Liver biopsy was done which showed congo red stain positive for amyloid. The patient's clinical condition continued to worsen and he was started on hemodialysis. During hospital course, the patient developed liver failure. His family members opted for palliative care and the patient passed away during the same admission. Physicians need to be aware of the detrimental course and poor prognosis associated with hepatic and renal amyloidosis. High clinical suspicion is needed to make an early diagnosis and initiate prompt treatment. Although clinical, laboratory and radiological findings can help in suggesting amyloidosis, a tissue biopsy is needed to confirm the diagnosis of amyloidosis.
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Introduction: Primary vaginal malignant melanomas are rare tumours with a limited number of cases published in the literature. They primarily affect post-menopausal women with a median age of 57-68 years and have a dismal prognosis. The 5-year survival rate, regardless of treatment, is approximately 5-25%. Case description: We present the case of an 87-year-old female who presented with haematuria and urinary incontinence. She was diagnosed with AJCC stage IIIC vaginal melanoma. Considering her age and the extent of malignancy, surgery was not a viable option and immunotherapy with nivolumab and ipilimumab was initiated as treatment. Discussion: The diagnosis of vaginal melanomas includes pathological analysis and immunohistochemistry (IHC) of the mass, imaging to determine extent, and genetic testing. Surgery is the preferred treatment in suitable cases. For metastatic or unresectable cases, immunotherapy or targeted therapy is the preferred first-line treatment. Due to the lack of an adequate number of cases to conduct randomized clinical trials, prognostic factors and treatment protocols for vaginal melanomas are not clearly defined. At present, the management of these tumours is largely based on retrospective studies and anecdotal evidence accompanied by significant knowledge gaps. Our case will be a valuable addition to the existing literature on vaginal melanomas that are managed non-surgically. LEARNING POINTS: Vaginal melanomas are extremely rare entities that require early diagnosis to ensure the best prognosis.Providers need to stress the importance of elderly gynaecological examination so crucial diagnoses are not missed.Further research is necessary to develop the most effective treatment plan for vaginal melanomas.
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Uterine leiomyosarcoma (ULMS) is an uncommon malignant tumor that accounts for less than one-third of uterine sarcomas and approximately 1% of uterine malignancies. Cases of local and distant ULMS metastasis have been widely reported, especially to the lungs. There have been very few cases, however, of ULMS metastasis to the upper gastrointestinal (GI) tract published in the peer-reviewed literature. Here we describe a case of biopsy-proven ULMS metastasis to the first part of the duodenum, representing the third reported case of its kind. Theories regarding presentation, treatment, clinical course, and outcome of patients with ULMS metastasis to the duodenum are presented.