Kunio Ichijima

Extranodal non-Hodgkin's lymphoma of the head and neck. A clinicopathologic study in the kyoto-nara area of japan

The clinicopathologic features of 114 Japanese patients with extranodal non‐Hodgkins lymphoma of the head and neck region were analyzed. The median age was 60.5 years and the male:female ratio was 1.5:1. The most common site of involvement was Waldeyers ring, followed by the oral cavity, thyroid gland, paranasal sinuses, nasal cavity, and larynx. Seventy‐five percent of the patients were in Stage I or Stage II at admission. Histologically, diffuse lymphoma accounted for 94% and follicular lymphoma for 6% of cases. The histologic grade according to the Working Formulation System of the National Cancer Institute was low in 11%, intermediate in 75%, and high in 14% of cases. Immunohistochemical study showed that the majority of the cases were of B‐cell type and only 13 cases (11%) were of the T‐cell type. Peripheral T‐cell lymphomas (eight cases) mainly occurred in the nasopharynx and nasal cavity, whereas four of five thymic T‐cell lymphomas were found in the palatine tonsil. The over‐all 5‐year survival rate was 54%, and the factors affecting survival were sex, histologic grade, T/B phenotype, clinical stage, and the site of initial presentation. Five‐year survival with nasal cavity and Waldeyers ring lymphoma was 24% and 46%, respectively. The poor prognosis of lymphomas at these sites might result from the predominance of T‐cell lymphoma, the paucity of low grade lymphoma, and the relatively high incidence of cases that were in an advanced stage at presentation. In Stage II, patients treated with combined therapy tended to have a better 5year survival rate than those treated with radiotherapy alone.

Malignant chondroid syringoma. Report of a case with widespread metastasis and review of pertinent literature

The authors report the autopsy case of a 73‐year‐old man with widespread, metastatic, malignant chondroid syringoma, with a long clinical history. Three years after resection of the primary tumor on the back, the tumor recurred at the same site, metastasized to the cervical lymph nodes 7 years later, and finally, after 13 years exhibited widespread metastases. Histologically, the tumor showed cords and nests of cuboidal or polygonal tumor cells with little cellular pleomorphism and few mitoses surrounded by a mucoid matrix. Histochemically, the matrix contained hyaluronic acid and sulfated acid mucopolysaccharides, such as chondroitin sulfate A and/or C. To the knowledge of the authors there have been nine reported cases of malignant chondroid syringoma in the English literature, four of which had distant metastasis. A review of the literature reveals that malignant chondroid syringoma is more common in women and occurs most often in trunk and extremities, which is in contrast to its benign counterpart, its histologic appearances vary greatly, and it may even have benign appearances.

PELIOSIS HEPATIS. An Unusual Case Involving Multiple Organs

The case is that of a 70‐year‐old Japanese male who had peliosis hepatis with disseminated peliotic lesions in various organs. The initial sign was spontaneous pneumothorax followed by pulmonary hemorrhage. There was no history of anabolic steroid ingestion and his history of pulmonary tuberculosis revealed no pathological changes. Autopsy findings suggested that dilatation of sinusoids with slow progressive injury of sinusoidal endothelium and hepatocytes due to unknown local and general etiologic factors may be an initial mechanism in the development of peliosis hepatis.

METASTASIS OF CANCER TO CANCER

A case of a 41 years old Japanese woman with widely disseminated primary lung cancer (adenocarcinoma) metastasizing to renal cell carcinoma (hypernephroma) is presented together with a brief review of the literature.

BREAST CANCER WITH REACTIVE MULTINUCLEATED GIANT CELLS: REPORT OF THREE CASES

We report here three cases of breast cancer with reactive multinucleated giant cells. The patients were among the 605 patients with breast cancer seen in the past 17 years at Tenri Hospital; the incidence of this variety of breast cancer was 0.5%. Enzyme histochemical and electron microscopic examination suggested that the giant cells were of histiocytic origin. However, results of immunohistochemical technique, S‐100 protein, lysozyme, nonspecific cross‐reacting antigen with carcinoembryonic antigen, α‐1‐antitrypsin, and α‐1‐antichymotrypsin, all currently used as markers of histiocytes, were negative. Because of the rarity of this variety of breast cancer, the biological significance of these unusual findings remains unknown.

An unusual case of metaphyseal chondrodysplasia with an abnormal perilacunar matrix associated with agranulocytosis and hypoplasia of the thymus

We report herein an unusual skeletal dysplasia in a 6-month-old boy characterized by metaphyseal dysplasia associated with agranulocytosis and hypoplasia of the thymus. A radiological survey revealed generalized metaphyseal abnormalities showing widening and increased density. Pathological changes in the chondro-osseous tissues were unusual and distinctive. There was widespread evidence of abnormal chondrocytes with an abnormal perilacunar matrix containing a PAS-positive, diastase resistant substance. Chondrocyte maturation and regular columnar arrangement were absent in most growth plates with only scattered ball-like nests of chondrocytes showing incomplete maturation. This case is a newly described form of osteochondrodysplasia.