A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure.

A 40-year-old male was hospitalized with renal impairment and severe hypercalcemia. His concentration of serum IgG4 was high, but serum whole PTH, 1-25(OH)2 vitamin D3 and PTHrP were not elevated. Computed tomography showed swelling of the bilateral lacrimal glands and systemic lymphadenopathy. The histological findings of lacrimal gland biopsy fulfilled the diagnostic criteria of IgG4-related ophthalmic disease (IgG4ROD). Bone scintigraphy showed increased ectopic uptake in the stomach, heart, lungs, and kidneys. He died on day 16 of admission, although the therapies for hypercalcemia were continued. Autopsy results showed an increase of osteoclasts in the bone marrow and metastatic calcification in multiple organs, and excluded from the differential diagnosis other disorders which present lymph-node swelling and hypercalcemia such as cancer, lymphoma, Castleman's disease, and sarcoidosis. He was given a diagnosis of IgG4ROD with osteolytic hypercalcemia.

[A Case of Successful Treatment of Recurrent Liver Metastases from Mixed Adenoneuroendocrine Carcinoma of the Pancreas with Everolimus Administration].

A 66-year-old man was admitted to undergo investigation for body weight loss. He was diagnosed with pancreatic neuroendocrine carcinoma, and a pancreaticoduodenectomy was performed. Three months after surgery, multiple recurrent liver metastases were observed on CT. We administered the cisplatin(CDDP)/irinotecan(CPT-11)regimen, but decided to discontinue the regimen because of adverse events. One month later, administration of everolimus(10mg/day)was initiated. Lesion loss was observed after 8 months, and the patient achieved a complete response(CR)without abnormal accumulation of FDG on PET-CT. Administration of everolimus was discontinued after 13 months because of peripheral neuropathy. Despite drug withdrawal, the patient has remained in remission for more than 3 years. Everolimus is considered an effective treatment for MANEC from NEC.

Diffuse Alveolar Hemorrhage in IgA Vasculitis with an Atypical Presentation.

IgA vasculitis (IgAV) commonly occurs in young children, who present with a tetrad of purpura, abdominal pain, arthralgia and nephritis. Diffuse alveolar hemorrhage (DAH) is a rare complication of IgAV. We herein report an adult case of IgAV with a presentation of DAH and nephritis (pulmonary renal syndrome, PRS), but without other typical manifestations, such as purpura, abdominal pain and arthralgia. A 33-year-old man presented with hemoptysis and a low-grade fever and was diagnosed to have IgAV based on the results of a renal biopsy. Treatment with corticosteroids, cyclophosphamide, and plasmapheresis was effective. IgAV should therefore be considered in the differential diagnosis of adult PRS.

Laparoscopic and Endoscopic Cooperative Surgery for Plexiform Angiomyxoid Myofibroblastic Tumor.

Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described distinctive gastric mesenchymal entity with a peculiar plexiform pattern, bland spindle cells and a myxoid stroma rich in arborizing blood vessels. In this study, we report a new case of this rare gastric tumor resected by laparoscopic and endoscopic cooperative surgery (LECS). A 39-year-old Japanese man was admitted with a gastric mass. Gastroscopy showed an elevated mass in the anterior wall of the gastric antrum. Endoscopic ultrasound examination revealed a focal hypoechoic lesion protruding into the lumen. A partial gastrectomy by LECS was performed, and the patient made an uneventful recovery and remains well 9 months later. The tumor in this case depicted all the typical histopathologic and immunochemical features of gastric PAMT (c-kit negative and smooth muscle actin-positive). Especially, it was characterized by multiple nodules protruding outward within the serosa. Therefore, it is important that the resection line is determined on the serosa to ensure the complete resection of these nodules together.