Pulmonary metastatic leiomyosarcoma coexisting with pulmonary chondroma in Carney's triad.

The diagnosis of Carney's triad requires the coexistence of at least two of three rare disorders, including gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma. Seventeen cases have been reported so far, occurring predominantly in young female patients. We report herein the 18th case of this entity and the first case, to our knowledge, where pulmonary metastases from a gastric leiomyosarcoma coexisted in a patient with benign pulmonary chondromas.

Minute pulmonary meningothelial-like nodules. A clinicopathologic study of so-called minute pulmonary chemodectoma.

The clinicopathologic features of 23 so-called minute pulmonary chemodectomas from nine patients are presented. Eight patients were women and one was a man; age range was 34-75 years (mean, 61). Two specimens were surgical resections and seven were autopsies (incidence, one per 60 autopsies). There was no association with a specific disease process or pathologic condition. Grossly, the lesions were 1-3 mm, tan to yellow, pleural or parenchymal nodules. Six of nine cases had multiple lesions; upper lobes were more often involved. Microscopically, characteristic cell nests expanded alveolar septa. Larger lesions were connected by intervening collagen, often imparting a stellate configuration. Smaller lesions had closely apposed nests with mildly thickened alveolar septa. The nodules were strongly reactive for epithelial membrane antigen (12 of 14) and vimentin (10 of 14), and were uniformly negative for cytokeratin, S-100, neuron-specific enolase, and actin. Ultrastructurally, complex interdigitating cell processes were connected by desmosomes. Occasional cytoplasmic filaments were seen. These nodules lack neuroendocrine features, differ from mesothelium, and strongly resemble meningothelial cells. A more accurate term for these lesions is minute meningothelial-like nodules. Their relationship to larger, solitary pulmonary meningiomas is unclear.

Pulmonary meningothelial-like nodules: a genotypic comparison with meningiomas.

BACKGROUND: Minute pulmonary meningothelial-like nodules (MPMNs) are incidental interstitial pulmonary nodules. They share histologic, ultrastructural, and immunohistochemical features with meningiomas (MGs). DESIGN: Sixteen cases yielding 33 separate MPMNs and 10 cases of benign MG were studied. Immunohistochemical studies and mutational analyses were performed on microdissected tissue using 20 polymorphic microsatellite markers targeting 11 genomic regions in an effort to identify genetic similarities of MPMN and MG. RESULTS: A total of 96.6% of MPMNs stained positive for vimentin, 33.3% for epithelial membrane antigen, 3% for S-100, and all were negative for cytokeratin and synaptophysin. Loss of heterozygosity (LOH) was identified in 25% of single MPMN affecting 3 genomic loci. No solitary MPMN had more than 1 LOH event. Multiple LOHs were seen only in MPMN-omatosis syndrome, where 33.3% of MPMNs showed LOH affecting 7 genomic loci. MG showed the highest frequency of LOH with major events seen at 22q (60%), 14q (42.8%), and 1p (44.4%) that were not shared by MPMN. CONCLUSION: Isolated MPMN lacks mutational damage, consistent with a reactive origin. MPMN-omatosis syndrome might represent the transition between a reactive and neoplastic proliferation. MPMNs are different from MG based on the major molecular genetic events seen in their formation and progression.

Pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma: Carney's triad.

The triad of pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma has been reported in five patients, all young women. The association of tumors apparently constitutes a syndrome. This paper describes the sixth affected patient, also a young woman, draws attention to the frequency of thoracic paraganglioma in the syndrome, and notes that the pulmonary cartilaginous lesion has frequently been misinterpreted as pulmonary hamartoma. Early exploration of the chest is indicated not only to rule out the possibility of metastatic gastric tumor but also to search for paragangliomas.

Melanotic paraganglioma of the posterior mediastinum.

A melanotic paraganglioma occurred in a 57-year-old woman, located in the left paravertebral space of the upper mediastinum. It was totally resected. During a 5 year follow up period neither tumour recurrence nor metastasis were observed. Histological examination of the tumour revealed a paraganglioma with monomorphous chief cell like elements which were arranged in a "zellballen" pattern. Immunohistochemical results also were in accordance with the diagnosis since neuron-specific enolase, chromogranin and synaptophysin were found in tumour cells whereas keratin was not. Additionally, neurosecretory granules were found in tumour cells during electron microscopy. A peculiar feature of the tumour was its strong pigmentation due to melanin located within the tumour cells and tumour associated melanophages. The simultaneous expression of functional properties of two different neural crest derived cells in one tumour stresses the close relationship between all neural crest elements and is in accordance with the observation of other melanotic, non-melanomatous tumours.

Ultrastructure of malignant paraganglioma of organ of Zuckerkandl.

Electron microscopic study of a malignant paraganglioma of the organ of Zuckerkandl revealed similarities between the tumor and the normal paraganglia. The well-differentiated portion of the tumor recapitulated the structure of the basic functional units of the paraganglion. In addition there signs of anaplasia both at the histologic and ultrastructural level. Large neurosecretory granules were noted in some of the tumor cells, but most of the cells were agranular. Crystaloids resembling those seen in alveolar soft part sarcoma were also noted. The study supports the theory on the common origin and histogenesis of paragangliomas and alveolar soft part sarcomas.

The neuroendocrine nature of the glomus cells: an experimental, ultrastructural, and histochemical tissue culture study.

Although the carotid body is an established chemoreceptor, there is considerable evidence also for its possessing a secretory function. While adrenergic neuroendocrine cells of neuroectodermal derviation exist in the central and autonomic nervous systems, the exact histogenesis of the mammalian carotid body is unsettled. The normal human carotid body and glomus jugulare tumor have been grown in tissue culture and their constituent cells have been observed to transform from epithelial to neuronoid appearing cells with extensive dendritic processes. This conversion has been further enhanced by the addition of nerve growth factor, a polypeptide specific for neural tissue. Electron microscopy confirmed that these culus cell. Histofluorescence revealed that these in vitro cells continued to synthesize and store biogenic monoamines in culture. Comparison of the morphologic, ultrastructural and histochemical features of the glomus cell with established neuroendocrine cells (central nervous system neurons, sympathetic ganglia cells, chromaffin cells) shows striking similarities. On the basis of these findings it is concluded that the glomus cell is a modified neuron of neural crest origin. The embryology, electron microscopy and histochemistry of the carotid body and related glomera and their tumors are reviewed.

Chemodectoma of the cauda equina: case report.

Well-differentiated paragangliomas have been found in unusual sites where conspicuous paraganglions are not normally found. A patient with a chemodectoma of the cauda equina is described. The importance of differentiating such tumors from those far more commonly seen in the region of the cauda equina, namely, ependymomas, hemangioblastomas, and metastatic carcinomas is emphasised.

Aortic body tumour with adjacent ectopic thyroid tissue in a dog.

A 12-year-old neutered male Husky dog had a neoplasm at the base of the heart which did not invade surrounding tissues. Microscopically, the neoplasm was composed of nests and sheets of polyhedral cells subdivided into lobules by trabeculae of fine fibrovascular stroma. Adjacent to the neoplasm was a rim of ectopic thyroid tissue that appeared histologically normal. The possible differential diagnoses for the neoplasm were aortic body tumour, ectopic thyroid tumour and ectopic parathyroid tumour; the ultrastructural characteristics revealed it to be an aortic body tumour.

An uncommon apudoma: a functional chemodectoma of the larynx. Report of a case and review of the literature.

The authors report a case of laryngeal chemodectoma in a 53-year-old man who died from general dissemination and lymphatic and subcutaneous metastases. This functional neoplasm secreted calcitonin revealed by fluorescence and biochemical tests and perhaps adrenaline-like substances. Its cells looked like the light chief cells of the human carotid body; they contained secretory granules and lysosomal formations. Thirty similar cases had been previously reported. The superior laryngeal glomi and more occasionally the inferior ones were the seat of these tumours which occurred with an equal frequency in both sexes. A hoarseness was often the unique symptom noticed during the several months or years preceding the first examination of this slow growing neoplasm. Nevertheless among the chemodectomas of the head and the neck region those arising in the larynx had the highest incidence of malignant evolution. Five of the patients presented a general dissemination, two had lymphatic metastases and one a local recurrence. The ultra-structural features and sometimes the rarely detected functional activity of these chemodectomas are those of apudomas whose cells might originate from the cephalic portion of the neural crests.

[A case of multiple chemodectoma: clinical and electron microscopical studies]. / Ein Fall des multiplen Chemodektoms. --Klinische und elektronenmikroskopische Untersuchungen--.

We observed electronmicroscopically and clinically a case of multiple chemodectoma, which has occurred from glomus caroticum sinistra and glomus jugularis sinistra of a 18 year old boy. The diagnosis was especially made by an angiography of the left common carotid artery and by a retrograde venography of the left internal jugular vein. Electronmicroscopically two kinds of tumor cells in this chemodectoma were identified. These cytoplasmas showed cored neurosecretory granules measured 125 to 250 A in diameter markedly increasing in number. Moreover in the island of the tumor cells of the carotid body tumor we happened to observe the nerve ending.

Paraganglioma: an unusual tumour of the parathyroid gland.

A case of paraganglioma arising within a parathyroid gland is reported. The lesion was an incidental finding in a block dissection of neck performed for squamous carcinoma of the pharynx. A well-circumscribed lesion, exhibiting the characteristic pathological features of a paraganglioma, was embedded within the right inferior parathyroid gland. Due to its location, the chief histological differential diagnosis was an unusual variant of parathyroid adenoma. Immunohistochemistry and electron microscopy assisted in reaching a diagnosis. This, as far as we are aware, is the first reported case of a paraganglioma of the parathyroid gland.

[So-called glomera in the region of the prostate]. / Sog. Glomera im Bereich der Prostata.

In three cases prostate biopsies revealed structures which showed the histological characteristics of so called glomera (chemodectomas), one within the prostatic tissue, the others in the area of the capsule. Clinicians and pathologists to avoid misinterpretation have to be aware of the fact that this wellknown structure can occur in biopsy material.

[Intrapulmonary chemodectoma. A new case with ultrastructural study]. / Chémodectome intra-pulmonaire. Un nouveau cas avec étude ultrastructurale.

The authors report the case of a 40-year old woman who presented with a round tumour in the middle lobe of the right lung. Once removed, the tumour proved to be an intrapulmonary chemodectoma. Chemodectomas are tumours that are rarely present in the lung. Their pathogenesis is open to discussion. Their study by electron microscopy is interesting, as it shows grains of neurosecretion and provides additional information on the morphology of chemoreceptors.

[Ultrastructure of chemodectomas of the neck]. / Ul'trastruktura khemodektom shei.

Two types of cells (dark and clear) with secretory granules in the cytoplasm were identified in these tumors. No specific ultrastructural features that would distinguish tumors differing in the degree of maturity were noted in the cells.

[Clinico-morphologic and electron microscopic characteristics of chemodectoma of the neck]. / Kliniko-morfologicheskaia i élektronno-mikroskopicheskaia kharakteristika khemodektomy shei.

Chemodectoma of the neck is one of the rare tumors occurring in man. The paper presents an observation of cervical chemodectoma found in a patient aged 31 years. For preoperative differential diagnosis, carotid angiography, ultrasound and radioisotopic examinations were used, which allowed the tumor located at the bifurcation of the left carotid artery to be diagnosed. The material taken during the surgery was histologically and electron microscopically examined to verify benign chemodectoma (an alveolar variant).

[Morphology and histogenesis of paraganglioma]. / Morfologiia i gistogenez paragangliomy.

Histological and ultrastructural studies were performed to examine 6 paragangliomas of various sites: carotid glomus, aortic body, sympathetic chain, and stomach. Five of the tumors in question had a histological structure typical of a paraganglioma. The gastric tumor was distinguished by being similar to a pheochromocytoma. With regard to the degree of infiltrative growth, the paragangliomas of the sympathetic chain and stomach were regarded as malignant. The ultrastructures of the paragangliomas examined were found to be of the same type. In all the neoplasms, tumor cells had signs of neuroendocrine and neurogenous differentiations that were characteristic of the ultrastructural organization of postganglionic neurons in the autonomic nervous system. The neurogenous differentiation was evidenced by cytoplasmic portions that are structurally similar to rough-surface endoplasmic reticulum, which is pertaining to Nissl's bodies; neurofilaments that form concentric structures containing neuroendocrine granules such as fibrous or Pick's bodies; intracytoplasmic cilia; specialized cytoplasmic processes of two types: axons and dendrites; partially reduced intercellular contacts, such as axosomatic and axodendritic synapses; sustentacular cells (sustenocytes). In the neoplasms evaluated as malignant, the neurogenous differential signs were more pronounced by reducing the number of neurosecretory granules, which might, apparently, serve as an ultrastructural criterion for establishing the degree of paraganglioma malignancy.

[Electron-radioautographic study of parasympathetic paragangliomas (chemodectomas) of the neck]. / élektronno-radiograficheskoeissledovanie parasimpaticheskikh paragangliom (khemodectom) shei.

Chemodectomas, after the surgery, were studied in 7 patients. Morphologically their malignancy is proven on the basis of pronounced cell polymorphism, atypia, invasion of tumour cells through the vessel wall into their lumen. Besides rare mitoses, proliferative (DNA synthesis) and protein synthetic (RNA synthesis) activity in clear and dark cells was not observed in the presence of intensive biosynthetic processes in the stroma cells.

[Electron microscopic diagnosis of granular cell tumors]. / Elektronno-mikroskopicheskaia diagnostika nekotorykh zernistokletochnykh opukholei

In tumour cells organspecific ultrastructural features are retained that may be of value in differential diagnosis. For this purpose not only fresh material but also that fixed formerly in formalin may be used.

[Histological variants of parasympathetic paragangliomas (chemodectomas) of the neck and morphological variants of their malignancy]. / Gistologicheskie varianty parasimpaticheskikh paragangliom (khemodektom) shei i morfologicheskie kriterii ikh zlokachestvennosti.

Histotopograms of 28 chemodectomas have been studied. Principal microscopic features of malignancy are as follows: structural atypia (complete in solid or fibrous variants) or partial (alveolar chemodectoma) loss of alveolarity as the most important sign of organotypia; another sign of malignancy is cell cataplasia (cell and nuclear polymorphism, change in nuclear-cytoplasmic relationship in the direction of its increase, nuclear hyperchromatism, appearance of large ungly hyperploid nuclei, mitotic activity of tumour cells in one case. Incomplete angiogenesis characteristic for malignant tumours was also detected. Invasive growth in the majority of chemodectomas in the form of partial growth through the walls of great vessels and ubiquitous growth through the walls of small vessels were present in most chemodectomas. Thus, there is a basis to claim that the chemodectomas studied are malignant tumours although they are characterized by a slow (years) growth and slow progression.