Parathyroid carcinoma is rare cause (less than 1%) of primary hyperparathyroidism [1]. Typically, higher serum levels of parathyroid hormone (PTH) are seen in parathyroid carcinoma (3 to 10 times, or even higher, the upper limit of the reference range) than in parathyroid adenoma and hyperplasia and are associated with extreme hypercalcemia (>14mg/dL) [1,2]. There may be associated severe bone pain and ossteitis fibrosa cystica [1]. Parathyroid carcinoma is an indolent tumor with low malignant potential and, in general, has a tendency to recur locally in the operative site or spread into contiguous tissues in the neck. Distant metastases are a late phenomenon with lymphatic or hematogenous spread to cervical lymph nodes and lungs and less commonly to the liv- , SHImeru kamIHara 2) anD kaTSumI egucHI er. The optimal treatment for parathyroid carcinoma is complete resection of the primary site, en bloc, including the surrounding tissues. When metastatic parathyroid tumors are found, surgical resection of the metastatic tumors is the optimal treatment. This is because parathyroid carcinoma is refractory to radiation and cytotoxic reagents [1]. medical management of hypercalcemia by infusing large amounts of fluid, along with bisphosphonates and perhaps calcitonin is briefly effective but, hypercalcemia eventually becomes uncontrolable. recently, cinacalcet, a calcimimetic agent [3], has been used effectively to reduce hypercalcemia in patients with primary hyperparathyroidism [4] and ~ two thirds of patients with parathyroid carcinoma also respond [5]. However, this treatment was not available in Japan for our patient in 2007, and, moreover, long-term efficacy has not been well established and calcimimetics do not inhibit proliferation of parathyroid cancer cells.It was recently shown that immunization with PTH peptides was effective in controlling hypercalcemia
IgG4-related sclerosing disease (IgG4-RSD) is an inflammatory and fibrosing disorder characterized by lymphoplasmacytic inflammation with infiltration of various organs, including the pancreas, bile ducts, lung, kidney, and retroperitoneum. As for malignancy in IgG4-RSD, only limited literature is available. We report here a case of thyroid papillary carcinoma showing unique morphology in IgG4-RSD. Solid tumor nests were surrounded by dense IgG4-positive plasma cells and fibrosis at both the primary site and metastatic lymph nodes. In contrast the background thyroid showed focal lymphocytic thyroiditis. IgG4-related sclerosing sialadenitis and autoimmune pancreatitis were also diagnosed, and prednisolone treatment improved symptoms and serum IgG4 abnormality. To the best of our knowledge, this is the first documentation of a malignancy of the thyroid gland occurring in a background of IgG4-RSD. A brief review of the literature on the relationship between IgG4 and malignancy is included.
Concurrence of nonalcoholic steatohepatitis (NASH) with autoimmune hepatitis (AIH) is a rare condition that is challenging to diagnosis, due to the relatively high prevalence of autoantibodies in NASH. It is also difficult to determine the most effective treatment as corticosteroids are likely to worsen NASH despite being effective in the treatment of AIH. In this case report, we present a female diagnosed with NASH-AIH overlap with accompanying diabetes mellitus, who successfully achieved normalization of serum alanine aminotransferase levels following prednisolone therapy and weight loss. A follow-up liver biopsy performed 40 months after the initial diagnosis showed only minimal inflammatory infiltrates in the portal area without any NASH histology. Resolution of NASH, in conjunction with a reduction in hepatic fibrosis, might suggest that prednisolone itself does not aggravate steatohepatitis, but rather prevents disease progression. Appropriate immunosuppressive treatment may therefore be an important component of the optimum therapy for NASH-AIH overlap.
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