肺嗜酸细胞类癌临床病理分析

目的探讨肺嗜酸细胞类癌的临床病理特点和鉴别诊断要点及生物学特征。方法对3例肺嗜酸细胞类癌组织病理学、免疫组化及电镜观察,并复习临床资料及相关文献。结果该肿瘤好发于男性,发病年龄16～80岁(平均49.9岁),临床症状以咳嗽、咳痰及咯血为主。组织学:肿瘤细胞呈卵圆形、多边形,胞质丰富,内见大量嗜酸性颗粒。免疫组化:肿瘤细胞表达神经分泌标记物NSE、Syn、CgA及CK阳性。电镜下胞质内见神经内分泌颗粒及较多肿胀的线粒体。随访3例中有2例癌转移。结论肺嗜酸细胞类癌是一类罕见的、具有独特形态特征的神经内分泌癌,诊断主要依靠组织学、免疫组化及电镜检查,其生物学行为类似于不典型类癌。     

Primary epithelioid malignant schwannoma of the lung

This is the first documented report of a case of primary malignant nerve sheath tumor of the lung showing an epithelioid malignant schwannoma. A 55 year old Japanese female underwent lobectomy of the middle lobe of the right lung because of a large, rounded tumorous shadow found on chest X-ray film. Light microscopic examination of the neoplasm disclosed an epithelioid malignant schwannoma with foci having a spindle-cell sarcomatous pattern. The neoplastic cells were positive for S-100 protein and neuron specific enolase by the immunoperoxidase method. Transmission electron microscopy showed basement membrane-like material surrounding the neoplastic cells and long spacing collagens in the interstitial spaces. The patient died of a recurrent tumor in the right hemithorax 5 months after undergoing lobectomy. An autopsy revealed no primary site of the neoplasm outside the lung. Furthermore, there was no evidence of neurofibromatosis.     

Gastric carcinoid tumors in a brother-sister occurrence, with special reference to ultrastructural features of endocrine granules

Our report concerns gastric carcinoid tumors in a man and his younger sister who had no endocrine symptoms. The tumors in both cases were multiple, located in the corpus, and showed no significant differences in light microscopic and immunohistochemical examination. In electron microscopic observation, the endocrine granules of tumor cells were roughly classified into four types: granules with fine granular content with low electron density, irregularly striped granules with medium electron density, concentrically laminated or regularly striped granules, or smaller and almost uniformly high electron-dense granules in which it was difficult to recognize the internal structure. From the sequential morphological changes of the granules, we have considered that each type of granule reflects various stages of granulogenesis, although the possibility that they are distinct and contain different hormones or chemical mediators is not absolutely ruled out.     

A light and electron microscopic study of an ovarian and rectal carcinoid

An ovarian and rectal carcinoid within the same person is described. The ovarian carcinoid is non-argentaffinic with typical regular granules. The rectal tumour consists of two types of APUD cells, some argentaffinic and diazo-positive, whilst others are non-argyrophilic. These features are mirrored ultrastructurally, some cells containing the large pleomorphic argentaffinic granules while others have smaller regular granules. In addition, focal aggregates of microfilaments were found in both tumour types. These findings are discussed in terms of the APUD concept. The similarities of the APUD tumour cells to D1 APUD cells of the duodenum is highlighted, and an alternative explanation for their derivation is proposed.     

Primary carcinoid tumor of the ear

Summary A very rare case of primary carcinoid tumor in the left ear of a 35-year-old woman is described. The argyrophilic property and uniformity of the size and shape of neurosecretory granules in the cytoplasm of tumor cells, correspond to the characteristics of carcinoid tumors derived from foregut endoderm. Clinical and light microscopic observations suggest this tumor originated from middle ear mucosa.     

Atypical carcinoid (neuroendocrine carcinoma) of the gingiva: counterpart of a laryngeal tumor

Intraoral localization of neuroendocrine carcinoma, usually called Merkel cell carcinoma, is extremely rare. A case of neuroendocrine carcinoma that was a counterpart of laryngeal neuroendocrine carcinoma but was not a Merkel cell carcinoma, occurring at the mandibular gingiva in a 69-year-old Japanese man, is described. The tumor formed a cauliflower-like mass, measuring 20 × 20 mm, with a small area of necrosis. A computed tomography image showed metastasis in the right submandibular lymph node. Histopathologically, the tumor was composed of immature, small round cells that formed anastomosing trabecular nests. Few mitotic and no necrotic features were observed in the nests. Immunohistochemical studies showed positive staining for chromogranin, synaptophysin and neuron-specific enolase in the tumor nests. We diagnosed it as an atypical carcinoid (neuroendocrine carcinoma), a counterpart to the same type of tumor occurring in the larynx. The present case is an extremely rare case of neuroendocrine carcinoma without the feature of Merkel cell carcinoma arising from the gingiva.     

Primary carcinoid tumor of the larynx and review of the literature

Primary carcinoid tumor of the larynx is very rare. This is the fifth case reported in the literature and the first autopsy case of laryngeal carcinoid. The early manifestation of the present case was multiple metastasis to the skin. At autopsy there was a laryngeal tumor associated with widespread visceral and cutaneous metastases. The tumor proved to be carcinoid tumor by histological, histochemical and electron microscopic findings.     

Microcystic variant of localized malignant mesothelioma accompanying an adenomatoid tumor-like lesion

The case of a 70-year-old man with a hitherto undescribed pleural mesothelioma is reported. The tumor was localized in the left lung apex and had invaded the parietal pleura. Histologically, the tumor was characterized by a proliferation of epithelioid cells and the formation of microcysts. The tumor cells were positive for calretinin and vimentin, and possessed abundant microvilli, indicating a mesothelial cell origin for the tumor. A high Ki-67 index and mitotic index, and the recurrence of the tumor after surgery, indicated malignancy. Based on the evidence, we propose that the tumor is a microcystic variant of a localized malignant mesothelioma.     

Primary carcinoid tumor of the kidney with special reference to its histogenesis

A case of primary carclnoid tumor of the kidney occurring In a 6&yeard women b reported. The tumor was 10 times 10 times 9 cm In she, did, yellowlsh-white In color, and associated with nuuslve hemorrhagk necrosis. Hlstoluglcally, it was composed of trebecular and anastornosing ribbonlike nests. The tumor cells showed argyrophllbity with the Grlmelius stain and cytoplasmlc PCwitMty for neumendocrlne markers, (Including chromogranln A, neuron-specifie endase, synap tophysln and Leu-7), prostetlc acid phosphatase, keratin and vimentln. Numerous cytoplasmlc neurosecretory granules were demonstrated uhstmcturally. These histologic and Immunohistochemical findings are consisten with the theory that this tumor has a common phenotype with that of carcinoid tumor aridng from the hlndgut or cloaca.     

Primary malignant melanoma of the rectum

The diagnosis of a primary rectal malignant melanoma has been the subject of considerable argument for many years, principally on the grounds that extension from a primary anal lesion was often impossible to exclude. This paper provides evidence that certain melanomas can be recognized as primary rectal tumours, not only from careful anatomical assessment of the site of the tumour, but also by showing that they arise on a background of benign melanocytic proliferation in the rectal mucosa. The clinical presentation of these lesions, their possible mechanism of origin and their treatment is discussed.     

Primary hepatic carcinoid and neuroendocrine carcinoma: Clinicopathological and immunohistochemical study of five cases

Primary hepatic carcinoid and neuroendocrine carcinoma (NEC) are rare tumors. We experienced three carcinoids and two NEC originating in the liver during the past 25 years and attempted to elucidate the clinicopathological and immunohistochemical features of these tumors. The patients had no endocrine symptoms despite two of them having elevated plasma serotonin. Three of the five patients died of the tumor after operation with an average survival time of 20.6 months. All tumors were large (up to 26 cm in diameter), four of them solitary and one multinodular, and were not associated with liver cirrhosis. The carcinoid tumors showed insular, trabecular or glandular arrangement of argyrophilic cells, whereas in the NEC this histological pattern was distorted. Immunohistochemically the tumors showed expression of chromogranin A (all cases), chromogranin B (three cases), pancreastatin and chromostatin (four cases, respectively), prohormone convertase PC3 (three cases), carcinoembryonic antigen (CEA) and CA19-9 (two cases), cytokeratin 56 kDa (three cases), 160 kDa neurofilament (two cases) and neuron-specific enolase (two cases). Serotonin and glucagon were sporadically detected in two tumors. The most useful marker to confirm the diagnosis was chromogranin A, which was cleaved to pancreastatin and chromostatin in the tumor tissue, and was more reliable than other markers of neuroendocrine differentiation.     

Histochemical and ultrastructural analyses of glandular differentiation in typical carcinoid tumor of the hindgut

Adenomatous differentiation of typical carcinoid tumors was examined by histochemical and ultrastructural studies on 33 cases of typical carcinoids of the hindgut. Of these 33 carcinoids, 12 (36%) showed a tubular and/or acinar pattern with production of mucus as a minor component. A few tumor cells with mucous globules were scattered among tubules in these carcinoids. Ultrastructurally, microvilli were seen along the luminal surface of tubules, with both numerous mucous globules in the supranuclear area and endocrine granules in basal sites of the same cells. These results indicate that even in typical carcinoid tumors, certain tumor cells have the features of goblet cells or the open type of endocrine cells. The findings of both mucous globules and endocrine granules in the same single cells indicate a close relationship between endocrine cells and mucus-producing and/or tubule-forming cells. It is concluded that pluripoten-tial ceils producing carcinoid tumors might be able to differentiate into three types of tumor cells: (i) endocrine cells without microvilli or mucus production; (ii) cells with microvilli and no mucus product; and (iii) mucus-containing cells with microvilli.     

An ultrastructural study of malignant fibrous histiocytoma of the larynx

Malignant fibrous histiocytoma (MFH) of the vocal cord occurring in a 46-year-old male was studied immunohistochemically and ultrastructurally. The tumor consisted of the two main areas, pleomorphic and storiform. Immunohistochemical staining was strongly positive for α1-antitrypsin and α1-antichymotrypsin, and negative for desmin, keratin, myoglobin, actin, carcinoembryonic antigen, and S100 protein. Ultrastructurally, this MFH consisted of four different types of cells which were either fibroblast-like, histiocyte-like, myofibroblast-like, or with features of both the fibroblast and histiocyte types. Transition forms between the four types of cells were also found. This suggests that cells of MFH are derived from the same undifferentiated stem cells.     

Primary carcinoid tumor of the testis: Immunohistochemical, ultrastructural and FISH analysis with review of the literature

Primary carcinoid tumor of the testis only accounts for 0.2% of all carcinoid tumors in Japan. The clinicopathological features, including differences between Japanese and Europe/United States (Eur/US) series', which are known in ovarian carcinoids, have not yet been sufficiently recognized. In the present study, five testicular carcinoids from various clinicopathological viewpoints were analyzed, with a review of the literature. All 15 cases in Japan, including 10 previously reported, were insular carcinoids. Three of the 15 cases (20%) were associated with a teratoma. Serotonin production was confirmed in at least 10 cases (five cases in the present study and five cases in previous studies), but carcinoid syndrome was present in only two cases. Three of the 15 cases (20%) showed distant metastases, and the tumors that metastasized were larger than those that did not. These features were almost similar to those observed in the Eur/US series. Three pure carcinoids were also studied for the number of sex chromosomes by fluorescence in situ hybridization (FISH). They did not show a significant numerical abnormality of the X chromosome, which is common in testicular germ cell tumors. Therefore, the genetic background of pure carcinoids might be different from that of common germ cell tumors.