Clinical, Pathologic and Immunohistochemical Study of Extramammary Paget's Diseases / 대한피부과학회지

BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon but distinctive tumor. Definitive diagnosis requires biopsy of the lesion and immunohistochemical staining. Immunohistochemical study using anti-cytokeratin monoclonal antibodies may be helpful for differentiating EMPD from other similar skin lesions and primary EMPD from pagetoid spread of underlying regional internal malignancy (secondary EMPD). OBJECTIVE: The purpose of this study was to identify clinical findings, histopathologic features and immunohistochemical characteristics of EMPD. METHODS: The twelve cases diagnosed as EMPD at our institution over ten years were included. Clinical records and pathology slides of the patients were reviewed retrospectively. Immunohistochemical stains for CK7, CK20, CEA, EMA, PSA, GCDFP15 and mucin stains such as PAS, d-PAS, alcian blue (pH 2.5) were done using paraffin blocks. RESULTS: 1. In most cases, EMPD developed on the scrotum of senile male patients. 2. In the pathologic findings, Paget cells were observed from the epidermis only in 2 cases, from the epidermis and intradermal skin appendages in 7 cases and from the dermis in 3 cases. 3. In all cases except for the single case associated with a transitional cell carcinoma of the urinary bladder, there were histochemical findings of alcian blue-, PAS- and immunohistochemical findings of CK7+, CK20-, CEA+, EMA+. 4. In the single case associated with transitional cell carcinoma of the urinary bladder, there were histochemical findings of alcian blue-, PAS- and immunohistochemical findings of CK7+, CK20+, CEA-, EMA-. CONCLUSION: The combination of CK7 and CK20 demonstrates these to be useful markers in distinguising primary EMPD from a pagetoid spread of extracutaneous malignancies. Namely, immunophenotypes other than CK7+/CK20- in Paget cells suggest underlying regional internal malignancy.

A Case of Primary Adrenal Gland Lymphoma

Primary adrenal lymphoma is extremely rare. We describe a case of non-Hodgkin's lymphoma of diffuse large B-cell type with right adrenal involvement. The patient received chemotherapy and external irradiation and achieved complete remission of the disease. We describe the case of primary adrenal lymphoma with a review of the literature on this unusual neoplasm. Primary adrenal lymphoma should be included in the differential diagnosis of adrenal mass.

A Case of Sporadic Nonfamilial Hypophosphatemic Osteomalacia / 대한내분비학회지

Acquired hypophosphatemic rickets, or osteomalacia, requires the recognition of the typical clinical and radiological features of osteomalacia in association with hypophosphatemia, which is caused by the decrease in intestinal absorption or impaired renal tubular phosphate reabsorption. The latter form may either be hereditary or acquired. Acquired hypophosphatemic osteomalacia includes oncogenic osteomalacia, neurofibromatosis, fibrous dysplasia, renal tubular acidosis and sporadic nonfamilial hypophosphatemic osteomalacia. A 33-year-old man presented with bone pain, progressive severe muscle weakness and a height loss of more than 10 cm over a 5 year period. The familiy history was negative for bone disease or other renal tubular defects. He was found to have hypophosphatemia, impaired phosphate reabsorption, normocalcemia, normal vitamin D metabolite levels, normal PTH and elevated alkaline phophatase. A bone biopsy showed thickened unmineralized osteoid compared to pelvic bone in control cases. Clinical symptoms, such as bone pain and muscle weakness, were improved after supplementation of oral phosphorus and calcitriol, although the serum phosphorus level did not normalize.