Recklinghausen病患者の頭部にみられた汗器官癌

抄録

A case of sweat gland carcinoma with von Recklinghausen's disease were reported. We considered this case syringoid eccrine carcinoma that had reported by Mehregan A.H., in 1983.
A 61-year-old man had a complaint of asymptomatic eruption on the vertex of the scalp on July 1991, that had increased in size slowly since resection of the traumatic scar on January 1990. Physical examination revealed a 15×15mm erosive lesion with crust on the vertex of the scalp and a lot of neurofibromas on the whole body. A 15×15mm red nodule was close to the scalp lesion.
Examination of the hematoxylin-and-eosinstained specimens showed many variously sized cysts extended in upper dermis and a lot of branching tubular structures embedded in a fibrous stroma of lower dermis. Tumor cells consisted by cystic and tubular structures were moderately atypical basaloid cells and did not form palisading. There was evidence of perineural and periarterial invasion. Evident connection between the tumors and neurofibromas was not found.
Immunohistochemical stain with Avidin-Biotinylated-Peroxidase-Complex method showed that luminal cells of cysts stained positive with carcinoembryonic antigen (CEA) and S-100 protein and tubular structural cells staind positive with CEA negative with S-100 protein. CAM5.2, antihuman cytokeratin, was reactive to the both structures. Vimentin showed negative activities.
Enzyme histochemical examination revealed tumor structures gave positive reactions for succinic dehydrogenase and negative for β-gluculonidase.
Electron microscopic findings were numerous intracellular ducts with prominent microvilli were present and tumor cells connected with desmosomes. Immature tonof filaments were found in some cytoplasmes.
In July 1991, the tumor was totally excised and skin-grafted. But the remnants of tumor cells were found at the margin with histological examination. In spite of a 40Gy electron radiation therapy, it recurred on previous skin graft in September 1991. The patient had wide surgical excision. It has not recurred in 11 months.
In 1983, Mehregan, studied 35 patients diagnosed in the past 20 years and classified eccrine adenocarcinomas the following 4 distinct variants histologjcally: eccrine porocarcinoma, syringold eccrine carcinoma, mucimous eccrine carcinoma and clear cell eccrine carcimoma.
Syringoid eccrine carcinoma (SEC) occurred on the scalp in four instances, on the extremities in four, on the back in two, on the ear and the cheek in each one. The lesions were single in all cases and measured from 1.5 to 4cm. Local lymphnode metastasis was rare.
Histologically, SECs were characterized by cystic and tubular structures resembled syringoma were composed by basaloid cells and invasively prolifetared in a fibrous stroma. Tubular structures were lined by a single layer of luminal cells and one or more rows of cells. Mitosis were often found. In some cases, tumor nests showed connections with the overlying epidermis. Perineural or perivascular invasion may be present. Enzyme histochemical showed positive reactions for succinic dehydrogenase and leucine aminopeptidase and negative for alkaline phosphatase.
Basal cell carcinoma has no activity to CEA that SECs react. Syringoma does not exhibit the aggressive infiltrative growth seen in the SECs. Cylindromas have more classic basaloid nests arranged in a jigsaw puzzle pattern. Primary cutaneus adenoid cystic carcinomas have its characteristic cribriform patterns.
We concluded our case differentiated into dermal duct through secretary portion by our immunohistochemical exmanination.
In Japan, 63 cases of non-neurogenic malignant neoplasms were reported among 2, 513 cases of von Recklinghausen's disease. It's rate was 2.5%. These complications may be accidental but a mutation theory is proposed that von Recklinghausen's disease patient may be given a tendency of occuring malignant neoplasms by gene mutation.