A case of adrenal myelolipoma complicated with Prader-Willi syndrome.

Introduction: Prader-Willi syndrome is a congenital disorder that occurs in one in 10 000-30 000 children and is characterized by obesity, short stature, and intellectual disability. Case presentation: A 24-year-old male patient with Prader-Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well-defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively. Conclusion: This is the first report of Prader-Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.

Myasthenic crisis appearing after resection of intracardiac ectopic thymoma with superior vena cava syndrome.

BACKGROUND: We describe herein an extremely rare case of intracardiac ectopic thymoma-only two pure cases have been reported to date-associated with myasthenia gravis, an infrequent complication of ectopic thymoma. CASE PRESENTATION: A 71-year-old woman with superior vena cava syndrome was found to have a large mass mainly located in the right atrium. Tumor resection under cardiopulmonary bypass was performed. The pathological diagnosis was type AB ectopic thymoma. The postoperative course was complicated by progressive respiratory failure, and she was diagnosed with myasthenic crisis based on clinical signs and the edrophonium test. The patient recovered and was weaned from prolonged mechanical ventilation after receiving intravenous immunoglobulin, and was subsequently discharged uneventfully. CONCLUSIONS: This is the first report of myasthenic crisis due to intracardiac ectopic thymoma. Residual thymoma is a risk factor for the development of post-thymectomy myasthenia gravis, and long-term follow-up is required.

[Immune thrombocytopenia associated with Kimura's disease].

In this study, we report a rare case of immune thrombocytopenic purpura (ITP) associated with the worsening of Kimura's disease. A 47-year-old Japanese man with a pruritic rash and swollen inguinal lymph nodes was diagnosed with Kimura's disease on performing a right inguinal lymph node biopsy. Thrombocytopenia ensued after the diagnosis of Kimura's disease, and fluctuations in the platelet count were observed along with the pathology of Kimura's disease. The platelet count fluctuated repeatedly with the relapse of Kimura's disease and a diagnosis of a combination of Kimura's disease and ITP was made through lymph node regeneration and bone marrow examination. Treatment with prednisolone (1 mg/kg/day) was initiated for Kimura's disease and ITP, and lymphadenopathy and platelet count improved promptly. Since then, the dose of prednisolone has been gradually reduced, and the disease status of both Kimura's disease and ITP has been controlled.

Pediatric plexiform fibromyxoma: A PRISMA-compliant systematic literature review.

BACKGROUND: Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with approximately 80 cases reported to date. Gastrointestinal stromal tumor, the most common primary mesenchymal tumor of the stomach, shows different biological and clinical characteristics between adult and pediatric patients. OBJECTIVES: This systematic literature review was conducted to elucidate the pathological and clinical features of pediatric PF compared to adult PF. METHODS: MEDLINE (1948 to March 2018) and EMBASE (1947 to March 2018) were searched, and all English articles that reported clinical data on PF patients were identified. Two authors independently reviewed the articles and extracted data to assess immunohistochemistry, sex, chief complaint, tumor size, tumor-related mortality, and tumor recurrence and metastasis. RESULTS: A total of 41 reports with 80 PF patients (of whom 70 were adult PF and 10 were pediatric PF patients) confirmed by histological and immunohistochemical findings were included. Of a total of 80 tumors, 62 (78%) were located in the gastric antrum, 42 (65%) presented with ulceration, and 48 (74%) were resected by partial gastrectomy. Median tumor size of the resected specimen was larger in pediatric PF than in adult PF cases (5.3 cm vs 4.0 cm, P = .036). However, there was no difference between pediatric and adult PFs in immunohistochemical expression, sex predominance, chief complaint, tumor-related mortality, and tumor recurrence and metastasis during the follow-up periods. CONCLUSION: Other than increased tumor growth in pediatric PFs, PF is a single disease entity with similar pathological features and benign clinical behavior regardless of onset age.

Promising biological therapies for ulcerative colitis: A review of the literature.

Ulcerative colitis (UC) is a chronic lifelong condition characterized by alternating flare-ups and remission. There is no single known unifying cause, and the pathogenesis is multifactorial, with genetics, environmental factors, microbiota, and the immune system all playing roles. Current treatment modalities for UC include 5-aminosalicylates, corticosteroids, immunosuppressants (including purine antimetabolites, cyclosporine, and tacrolimus), and surgery. Therapeutic goals for UC are evolving. Medical treatment aims to induce remission and prevent relapse of disease activity. Infliximab, an anti-tumor necrosis factor (TNF)-α monoclonal antibody, is the first biological agent for the treatment of UC. Over the last decade, infliximab and adalimumab (anti-TNF-α agents) have been used for moderate to severe UC, and have been shown to be effective in inducing and maintaining remission. Recent studies have indicated that golimumab (another anti-TNF-α agent), tofacitinib (a Janus kinase inhibitor), and vedolizumab and etrolizumab (integrin antagonists), achieved good clinical remission and response rates in UC. Recently, golimumab and vedolizumab have been approved for UC by the United States Food and Drug Administration. Vedolizumab may be used as a first-line alternative to anti-TNF-α therapy in patients with an inadequate response to corticosteroids and/or immunosuppressants. Here, we provide updated information on various biological agents in the treatment of UC.