Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern

Nicholson, Andrew G.; Baandrup, Ulrik; Florio, R; Sheppard, Mary N.; Fisher, Cyril

doi:10.1046/j.1365-2559.1999.00724.x

Cited by 48 publications

(33 citation statements)

References 11 publications

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“…It expresses S100 protein but not other melanocytic markers. PPMS, however, a rare pulmonary tumor recently described by our group, 19,29 which is sited predominantly endobronchially and characteristically harbors EWSR1-CREB1 fusion, 29 may cause diagnostic confusion with pulmonary AFH. PPMS is composed of cords and clusters of bland spindle to ovoid cells, often with a reticular pattern within prominent myxoid stroma.…”

Section: Discussionmentioning

confidence: 94%

Endobronchial Pulmonary Angiomatoid Fibrous Histiocytoma

Thway

Nicholson

Wallace

et al. 2012

American Journal of Surgical Pathology

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Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biological potential, predominantly occurring in the extremities of children and young adults. It has only recently been reported as a primary lung tumor. We describe 2 cases arising endobronchially harboring EWSR1 gene rearrangements by fluorescence in situ hybridization and, respectively, EWSR1-CREB1 and EWSR1-ATF1 gene fusions by reverse transcription polymerase chain reaction. Histologically, both tumors showed classical features of AFH, comprising multiple nodules of bland spindle to epithelioid cells surrounded by lymphoplasmacytic inflammation and at least a partial fibrous capsule. Both tumors showed focal but strong desmin immunoreactivity, with focal pancytokeratin and epithelial membrane antigen in 1 case. The lung is now a recognized site of AFH occurrence, but tumors arising here can be associated with different gene fusions. It is important to recognize AFH in the pulmonary region, as its behavior at other sites is generally relatively indolent; however, it may be mistaken for metastatic or more aggressive primary lung tumors. It is likely that cases of AFH in the lung may have been previously missed because of their morphologic and genetic overlap with other pulmonary lesions.

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Section: Discussionmentioning

confidence: 94%

Endobronchial Pulmonary Angiomatoid Fibrous Histiocytoma

Thway

Nicholson

Wallace

et al. 2012

American Journal of Surgical Pathology

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“…Two recent articles describe rare examples of malignant myxoid pulmonary tumors of uncertain histogenesis showing no or little evidence of chondroid differentiation. 5,13 The authors speculate that these malignant myxoid tumors may fall within a spectrum of extraskeletal myxoid chondrosarcoma or malignant mixed tumors of bronchial derivation. In the present cases, these considerations seem less likely, mostly due to innocuous cytology and benign clinical course.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Microcystic Fibromyxoma: Report of 3 Cases

Shilo

Miettinen

Travis

et al. 2006

American Journal of Surgical Pathology

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Three cases of pulmonary myxoid tumors showing a variable degree of microcystic change are described. They are presented as incidental solitary peripheral lung nodules on routine radiologic studies. The patients included 2 women, 45 and 65 years old, and 1 man, 33 years old. Clinical work-up showed no evidence of neoplasia in other sites. Following surgical resection, no evidence of disease was observed after a mean follow-up of 48 months. The tumors ranged in size from 1 to 2.3 cm (mean 1.4 cm). Microscopically, the lesions were well circumscribed with conspicuous cystic change and myxoid stroma. Innocuous, widely-spaced, spindled to stellate tumor cells showed minimal nuclear pleomorphism and absence of mitotic activity. The myxoid stroma contained acid mucopolysaccharides, sensitive to hyaluronidase digestion. No epithelial, chondroid, neural, myofibroblastic, lipomatous or vascular differentiation was evident on immunohistochemical studies. Although these cases display cytologic features, myxoid stroma and benign clinical course characteristic of pulmonary myxomas; the presence of microcystic architecture is unique to the current series, and thus a descriptive designation "microcystic fibromyxoma" is suggested.

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“…64 Primary pulmonary myxoid sarcomas are lobulated tumors composed of clusters and cords of largely bland spindle or ovoid cells, often with a reticular pattern within prominent myxoid stroma, which is Alcian blue positive and sensitive to treatment with hyaluronidase. 64,65 Primary pulmonary myxoid sarcomas bear a striking histologic resemblance to extraskeletal myxoid chondrosarcoma but lack the characteristic translocations of extraskeletal myxoid chondrosarcoma that fuse NR4A3 on chromosome 9q22 to a variety of partner genes. [66][67][68][69][70][71][72][73] Primary pulmonary myxoid sarcomas generally express only vimentin but are occasionally weakly and focally immunoreactive for epithelial membrane antigen.…”

Section: Geneticsmentioning

confidence: 99%

Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics

Thway

Fisher

2015

Archives of Pathology &Amp; Laboratory Medicine

117

134

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Context.-Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biologic potential and uncertain differentiation, most often arising in the superficial extremities of children and young adults. While it has characteristic histologic features of nodular distributions of ovoid and spindle cells with blood-filled cystic cavities and a surrounding dense lymphoplasmacytic infiltrate, there is a significant morphologic spectrum, which coupled with its rarity and lack of specific immunoprofile can make diagnosis challenging. Angiomatoid fibrous histiocytoma is associated with 3 characteristic gene fusions, EWSR1-CREB1 and EWSR1-ATF1, which are also described in other neoplasms, and rarely FUS-ATF1. Angiomatoid fibrous histiocytoma is now recognized at an increasing number of sites and is known to display a variety of unusual histologic features.Objective.-To review the current status of AFH, discussing putative etiology, histopathology with variant morphology and differential diagnosis, and current genetics, including overlap with other tumors harboring EWSR1-CREB1 and EWSR1-ATF1 fusions.Data Sources.-Review of published literature, including case series, case reports, and review articles, in online medical databases.Conclusions.-The occurrence of AFH at several unusual anatomic sites and its spectrum of morphologic patterns can result in significant diagnostic difficulty, and correct diagnosis is particularly important because of its small risk of metastasis and death. This highlights the importance of diagnostic recognition, ancillary molecular genetic confirmation, and close clinical follow-up of patients with AFH. Further insight into the genetic and epigenetic changes arising secondary to the characteristic gene fusions of AFH will be integral to understanding its tumorigenic mechanisms.

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Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern

Cited by 48 publications

References 11 publications

Endobronchial Pulmonary Angiomatoid Fibrous Histiocytoma

Endobronchial Pulmonary Angiomatoid Fibrous Histiocytoma

Pulmonary Microcystic Fibromyxoma: Report of 3 Cases

Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics

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