Ectopic adrenocorticotropic hormone hypersecretion due to a primary pulmonary paraganglioma.Endocr Pract. 2004 Sep-Oct;10(5):424-8.

OBJECTIVE: To present a rare case of primary paraganglioma of the lung, which caused the syndrome of ectopic adrenocorticotropic hormone (ACTH) hypersecretion. METHODS: The clinical, biochemical, and imaging findings in this case are described, and the pathologic features of the resected tumor tissue are depicted. In addition, the related literature on paragangliomas is reviewed. RESULTS: In a 39-year-old woman with hypertension, weight gain, and easy bruising, laboratory studies showed hypercortisolemia, high plasma ACTH levels, and suppression of cortisol by high-dose dexamethasone. An indium-labeled octreotide whole-body scan disclosed a tumor in the left lower lung field. Thoracoscopic wedge resection of the pulmonary mass was performed. Postoperative microscopic findings and immunohistochemical stains revealed nests of rounded and polyhedral cells and S-100 protein-positive sustentacular (supporting) cells, charac-CONCLUSION: This unusual case of Cushing's syndrome was attributable to an ACTH-secreting primary pulmonary paraganglioma.

Primary paraganglioma of the lung.Ann Diagn Pathol.2004 Aug;8(4):237-41

There are few reported cases of primary pulmonary paraganglioma in the pathology literature. Given the historical confusion surrounding bronchial tumors, widespread use of the term "chemodectoma" and classification of these lesions as paraganglioma in an outdated World Health Organization classification of lung tumors, the recognition of tumors arising from paraganglia within the lung has not been accepted by leading authorities. We present a well-documented case of a primary pulmonary paraganglioma with typical morphologic features and a supporting immunohistochemical profile. The 0.9 cm endobronchial tumor was submucosal and composed of nests of ovoid cells with abundant eosinophilic cytoplasm, cytoplasmic vacuoles, round to oval nuclei with speckled chromatin, and occasional conspicuous nucleoli. The nests of cells were surrounded by thin-walled vascular channels and stellate spindle cells. The ovoid cells showed strong diffuse staining for chromogranin A, synaptophysin, and faint staining for S-100; they were negative for cytokeratin AE1/AE3, Cam 5.2, and epithelial membrane antigen. The stellate spindle cells stained intensely positive for S-100 protein. A critical review of reported cases of pulmonary chemodectomas and paragangliomas in the English literature features few, if any, well-documented examples. While this exceedingly rare tumor should be discerned from carcinoid tumor, it remains unknown if primary pulmonary paragangliomas behave aggressively like intra-abdominal extra-adrenal paragangliomas, or in a more indolent manner observed with extra-adrenal paragangliomas in other locations.

Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study.Am J Surg Pathol. 2004 Jun;28(6):825-9.

We describe a case of primary pulmonary paraganglioma, a tumor that has not been reported in sufficient detail in previous literature. The patient was a 55-year-old woman with hypertension accompanied by an elevated serum norepinephrine level (2651 pg/mL; normal 100-450 pg/mL). Computed tomography revealed a well-circumscribed solid mass, 3.5 cm in diameter, located in the lower lobe of the left lung. In the lobectomy specimen, the tumor had invaded the B8 bronchus and hilar lymph nodes with microscopic metastasis to the mediastinal nodes. The tumor showed histologic, immunohistochemical, and ultrastructural features of paraganglioma: argyrophilic cells arranged in a nesting (Zellballen) or anastomosing trabecular pattern within an arcuate vascular network. Neoplastic chief cells positive for neuroendocrine markers (CD56, synaptophysin, chromogranin A) were surrounded by sustentacular cells positive for S-100 protein. Neurofilament protein was positively stained, but cytokeratins were totally negative. On electron microscopy, chief cells possessed abundant dense core granules with an eccentric halo ("norepinephrine-type" granules). The patient's blood pressure began to decline soon after the resection, and her serum norepinephrine promptly returned to almost normal. On the basis of our experience, our case is a bona fide primary pulmonary paraganglioma, a tumor heretofore subject to considerable skepticism.

Primary pulmonary paraganglioma.Zhonghua Yi Xue Za Zhi (Taipei). 2002 Sep;65(9):446-9.

We describe a 46-year-old man with a mass in the lower lobe of the right lung. The tumor was initially suspected to be a poorly differentiated adenocarcinoma after endobronchial biopsy. Subsequently, a right lower lobectomy was performed, and a paraganglioma was diagnosed histologically. Using whole-body CT scan and metaiodobenzylguanidine (MIBG) scintigraphy, we excluded the involvement of other organs. No evidence of recurrence or metastasis was found during the follow-up period of 3 years. Primary pulmonary paragangliomas are very uncommon tumors. Literature relevant to this disease entity is discussed.

Spindle cell carcinoids of the lung with paraganglioid features: a reappraisal of their histogenetic origin from paraganglia using immunohistochemical and electronmicroscopic techniques.Ultrastruct Pathol. 2001 May-Jun;25(3):207-17.

Five cases of spindle cell carcinoids of the lung were analyzed by immunohistochemical and ultrastructural technique. They were found to be biphasic tumors composed of the major component of neuroendocrine cells (chief cells) and a minor component of dendritic cells (supporting cells). The chief cells displayed positivity for neuroendocrine phenotypic antigenic markers: neuron specific enolase (NSE), chromogranin A, and synaptophysin. They contained varying numbers of dense-core granules by electron microscopy. In addition, the chief cells expressed cytoplasmic positivity for cytokeratins. The supporting cells were dendritic in appearance and displayed strong positivity for S-100 protein in all cases. Glial fibrillary acidic protein was positive in two cases. On electron microscopy, the supporting cells were agranular and found along the external lamina surrounding the nests of tumor cells. In two cases, rare ganglion cell-like cells were present. The histomorphologic, immunohistochemical, and ultrastructural features were contrastingly different from the classical pulmonary carcinoid and rather resembled gangliocytic paragangliomas arising from small intestine and spine. It is proposed that pulmonary carcinoids with biphasic features are better designated as gangliocytic paragangliomas of the lung rather than paraganglioid carcinoids.

Fine needle aspiration cytology of primary pulmonary paraganglioma. A case report. Acta Cytol. 2001 May-Jun;45(3):459-64.

BACKGROUND: Primary pulmonary paragangliomas are rare tumors. To our knowledge, there is no prior report on fine needle aspiration cytology (FNAC) in pulmonary paraganglioma. CASE: A 34-year-old man presented with an incidentally found solitary pulmonary mass. FNAC showed papillarylike clusters of epithelioid cells with round to oval nuclei, evenly dispersed chromatin, micronucleoli and occasional anisonucleosis. These cytologic features were suggestive of a sclerosing hemangioma or bronchioloalveolar carcinoma. A right lower lobectomy revealed a primary pulmonary paraganglioma. CONCLUSION: The possibility of pulmonary paraganglioma should be considered in the differential diagnosis of FNAC showing pseudopapillary clusters of epithelioid cells.

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.Am J Surg Pathol. 2001 May;25(5):688-93.

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.

An extremely large solitary primary paraganglioma of the lung: report of a case.Surg Today. 1999;29(11):1195-200.

We present herein the case of a 38-year-old woman found to have an extremely large solitary primary paraganglioma of the lung. The patient presented with chest pain on exertion and a mass was discovered in the left lower lobe of the lung by chest X-rays and computed tomography (CT). As no other neoplasms were detected elsewhere, a left lower lobectomy was performed. The patient has remained well without any evidence of recurrence for 5 years since her operation. The tumor, measuring 13 x 12 x 7 cm, was composed of ovoid cells (Zellballen), which were positive for Fontana-Masson and Grimelius stains, and sustentacular cells. Immunohistochemically, the ovoid cells were positive for neuron-specific enolase, S-100, CAM5.2, Leu7, and chromogranin A, and negative for carcinoembryonic antigen and epithelial membrane antigen. The sustentacular cells were positive for S-100 protein and CAM5.2, and negative for glial fibrillary acid protein. Therefore, the tumor was diagnosed as a paraganglioma. The tumor from our patient is the largest of the 17 solitary primary pulmonary paragangliomas reported thus far in the English-language literature.

Multiple pulmonary chemodectomas in a child: results of four different therapeutic regimens.J Pediatr Hematol Oncol. 1998 Nov-Dec;20(6):583-6.

PURPOSE: Chemodectomas (or paragangliomas) are rare tumors of neuroendocrine chemoreceptors, such as the carotid body. This report describes a case of multiple pulmonary chemodectomas in an adolescent and discusses the results of four therapeutic regimens. PATIENTS: At 15 years of age, the patient had cough and fatigue. Investigation revealed numerous 1- to 2-cm diameter nodules throughout both lungs. Biopsy revealed multiple pulmonary chemodectomas of uncertain malignant potential. No extrapulmonary primary site could be found. RESULTS: Because of deteriorating pulmonary function, she was treated with courses of etoposide-cisplatin and subsequently somatostatin without effect. She finally responded to a course of doxorubicin and streptozocin. She is currently maintained on interferon-alpha 2B but her measured vital capacity continues to fall slowly, reflecting increased tumor growth. Because there is still no evidence of extrapulmonary spread, she is considered to be a candidate for lung transplantation. CONCLUSIONS: A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas.

Multicentric metachronous pulmonary and intravagal paraganglioma: a case report with immunohistochemical findings.Arch Pathol Lab Med. 1996 Dec;120(12):1137-40.

An unprecedented presentation of multicentric paraganglioma in a 48-year-old man is described. One of the paragangliomas, originally diagnosed as a carcinoid tumor, presented as a lung mass and was removed. Four years later, an intravagal paraganglioma was discovered. The lung and intravagal tumors had identical morphologic and immunoreactive characteristics. Both tumors consisted of chief cells (type 1) and sustentacular cells (type 2). The chief cells were immunoreactive with neuroendocrine markers (synaptophysin and chromogranin), but nonreactive with epithelial markers (CAM 5.2, high- and low-molecular-weight keratins, epithelial membrane antigen, and carcinoembryonic antigen). The sustentacular cells were positive for S100 protein. Although pulmonary carcinoids may mimic paragangliomas and occasionally contain sustentacular cells, the diagnosis was rejected because the tumor cells did not demonstrate reactivity with epithelial markers.

Primary paraganglioma of the lung. Report of two cases and review of the literature. APMIS. 1995 Jul-Aug;103(7-8):597-603.

Two cases of primary paragangliomas of the lung are presented. The first occurred in a 69-year-old woman and was a grossly and histologically benign tumour. The other case occurred in a 33-year-old woman who had metastases to the peribronchial lymph nodes. This seems to be the second malignant case reported. No evidence of recurrence nor metastatic disease was found at follow-up, 8 months for the first case and 7 years for the second, respectively. The morphological and immunohistochemical findings are discussed and the literature comprising 20 cases is reviewed.

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