Noboru Onoda

Pigmented Dermatofibrosarcoma Protuberans (Bednaf Tumor)

An autopsy case of pigmented dermatofibrosarcoma protuberans (Bednaf tumor) with systemic metastasis is reported. No previous example of this tumor showing widespread metastasis has been reported in the literature. The patient, a 45 year old man, developed a tumor on the right upper arm. The tumor recurred twice and metastasized to other parts of the skin, lungs and brain during the 8‐year clinical course. The primary tumor contained melanin‐laden tumor cells and showed a storiform growth pattern. Autopsy confirmed multiple metastatic lesions in the skin, lungs, brain, thyroid, pancreas, stomach, small intestine and thigh muscles. The recurrent and metastatic tumors lacked both melanin production and the storiform arrangement, and instead revealed “fibro sarcomatous” change with a herring bone or interlacing pattern of growth. Acta Pathol Jpn 40: 935–940, 1990.

Immunohistochemical Demonstration of Nonspecific Cross‐reacting Antigen in Normal and Neoplastic Human Tissues Using a Monoclonal Antibody: Cornparison with Carcinoembryonic Antigen Localization

Nonspecific cross‐reacting antigen (NCA) immunoreactivity was localized in normal and neoplastic human tissues using a monoclonal antibody to 55, 90 and 95 kDa molecules of NCA. This was compared to the localization of immunoreactive carcinoembryonic antigen (CEA) as demonstrated by polyclonal and monoclonal antibodies. In frozen sections, CEA was localized in normal surface epithelium of the stomach and colon where NCA was only weakly detected. Type 1 and type 2 like pneumocytes were positive for NCA, while CEA was localized only in type 2‐like pneumocytes. CEA and NCA were both demonstrated in ductal cells of frozen pancreatobiliary and mammary tissues. The antigenicity of CEA and NCA in normal tissues was significantly lost after paraffin embedding as compared to frozen sections. NCA was consistently demonstrated in eccrine sweat glands embedded in paraffin. In various tumor tissues, CEA and NCA were colocalized and expression increased sufficiently to be detected in paraffin sections. Adenocarcinomas of the stomach and colon and cystadenocarcinoma of the pancreas, as well as neuroendocrine carcinomas of the lung and thyroid, showed a CEA predominance over NCA. In ductal adenocarcinomas of the pancreas and breast and in cholangiocarcinoma, NCA reactivity was greater than CEA. Keratiniring foci of most squamous cell carcinomas of mucosal origin and some adenocarcinomas equally expressed both. Hepatocellular carcinoma, lobular mammary carcinoma and papillary thyroid carcinoma were positive only with unabsorbed polyclonal antibody which widely recognizes CEA‐related substances. Renal cell carcinoma, prostatic adenocarcinoma, transitional cell carcinoma, anaplastic carcinomas, choriocarcinoma and basal cell carcinomas showed little or no immunoreactivity. Hence the relative ratio of CEA/NCA expression in tumors was dependent on the tissue of origin and histologic type. The cytoplasmic granular staining of NCA in cancer cells was a noteworthy difference from the plasma membrane‐associated localization of CEA. Acta Pathol Jpn 40: 85–97, 1990.

An Autopsy Case of lnvasive Pituitary Adenoma (Prolactinoma) with Rapid Fatal Clinical Course Due to Streptococcal Meningitis

A 44‐year‐old male suffered epistaxis and headache of sudden onset and was diagnosed as having suppurative meningitis due to streptococci. Four days after the onset of symptoms, he died despite treatment with antibiotics. Destruction and ballooning of the sella turcica was revealed by a plain head X‐ray examination during the clinical course. At autopsy, a massive tumorous lesion extended from the ballooned sella turcica to the paranasal cavities, nasopharynx and facial bone, and this had resulted in suppurative meningitis. The tumor was also disseminated to the basal skull. The tumor cells possessed prominent nuclear atypia and were immunohistochemically positive for prolactin. This was diagnosed as a case of pituitary adenoma with markedly invasive pathological findings and a rapid and fatal clinical course. Acta Pathol Jpn 42: 832–836, 1992.

Immunohistochemical localization of catecholamine-synthesizing enzymes in human pheochromocytomas

A total of 17 (10 adrenal and 7 extraadrenal) pheochromocytomas were examined immunohistochemically for the localization of catecholamine-synthesizing enzymes-tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and phenylethanolamine N-methyltransferase (PNMT)-as well as the marker peptides for pheochrornocytomas (i.e., met-enkephalin-arggly-leu [MEAGL]). Normal adult, fetal, and newborn human adrenal medullas were also examined. Six adrenal pheochromocytomas showed immunohistochernically positive staining for PNMT; 5 of these cases demonstrated elevated serum adrenalin levels, This indicated morphofunctional correlation. PNMT-positive cells were mostly positive for TH but did not show co-localization of MEAGL except in 1 case, Absence of co-localization was considered to reflect the physiological condition of the specimen, based on the similar staining in the normal adult adrenal medulla. Primary culture and immunoelectron microscopy suggested the processing and synthesis of adrenalin and MEAGL in the secretory granules.Seven extraadrenal pheochromocytornas were negative for PNMT and suggested the lack of adrenalin synthesis.

Malignant paraganglioma of the retroperitoneum with lung metastases: a 13-year survivor after radical surgery.

We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.

Tracheoesophageal anastomosis for intractable aspiration pneumonia.

Permanent tracheostomy and tracheoesophageal anastomosis were performed as a means of surgical intervention for the treatment of intractable aspiration pneumonia. Conventional methods of tracheoesophageal anastomosis have entailed various problems. The improved method devised by us uses the special histological features and enables safe and reliable anastomosis. By this method, the tracheal perichondrium is retained and the strength of the anastomosed portion of the trachea is maintained, the failure of sutures due to tension on the anastomotic site being prevented. Also, reanastomosis can be performed after the cure of primary disease.

A case of large cell carcinoma consisting of clear cell element.

明細胞が主体に構成される肺大細胞癌の1例を報告する.症例は86歳の男性.増量する喀疾を主訴に近医を受診し, 胸部X線写真上左肺上葉にcoin lesionを指摘された.気管支鏡下の気管支擦過細胞診を行いclassVと診断され, 左肺上葉切除術を施行された.気管支擦過細胞診所見は, 大小不同の著しい腫瘍細胞が, 比較的平面的に出現し, N/C比のばらつきも目立った.粗穎粒状のクロマチンを有し, 核小体の目立つ核と, ライト緑淡染性の淡明な細胞質を示した.切除肺には, 肉眼的に径3cmの黄白色境界明瞭な充実性腫瘍がみられた.病理組織所見では, 血管豊富な充実性腫瘍で, 明らかな扁平上皮癌および腺癌への分化は認めなかった.主体をなす細胞は, 細胞および核の大小不同が目立ち, グリコーゲンの豊富な明るい細胞質が特徴的であった.核の大小不同が非常に目立つ点と併せて, 細胞所見は組織所見をきわめてよく反映しているものと思われる.本邦における肺癌取扱い規約では, 明細胞癌は独立した組織型の存在が確認し難いとの理由で分類からは除外されている.WHO分類では肺明細胞癌は, 大細胞癌の一亜型として分類されており, その構成成分が淡明ないし泡沫状の胞体を有する腫瘍細胞であり, 腺細胞ないし扁平上皮への分化が認められていないものとして定義されており, そこに示されている基準を本例は満たしていると考える.腎細胞癌の肺転移を始め, 良性明細胞腫が鑑別診断として問題となったが, いずれも臨床検査上, また免疫組織化学的検索にて否定された.