Primary localised pleural neurofibroma: expanding the spectrum of spindle cell tumours of the pleura

Langman, Gerald; Rathinam, Sridhar; Papadaki, L

doi:10.1136/jcp.2009.067264

Cited by 9 publications

(4 citation statements)

References 13 publications

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“…In NF1, the thorax and lungs can be affected in several ways, given the dense distribution of peripheral nerves throughout the thorax: cutaneous and subcutaneous neurofibromas on the chest wall and chest wall deformities; kyphoscoliosis; ribbon deformity of the ribs; thoracic neoplasms; parenchymal neurogenetic tumors [29-31]; and other respiratory manifestations that severely impair pulmonary function, such as upper airway obstruction by neurofibromas, central hypoventilation, diaphragm paralysis, diffuse and interstitial lung disease, and primary pulmonary hypertension [32-35]. …”

Section: Discussionmentioning

confidence: 99%

Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

Masocco

Kodra²,

Vichi

et al. 2011

Orphanet J Rare Dis

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BackgroundPersons affected by neurofibromatosis type 1 (NF1) have a decreased survival, yet information on NF1-associated mortality is limited.Methods/AimThe National Mortality Database and individual Multiple-Causes-of-Death records were used to estimate NF1-associated mortality in Italy in the period 1995-2006, to compare the distribution of age at death (as a proxy of survival) to that of the general population and to evaluate the relation between NF1 and other medical conditions by determining whether the distribution of underlying causes of NF1-associated deaths differs from that of general population.ResultsOf the nearly 6.75 million deaths in the study period, 632 had a diagnosis of NF1, yet for nearly three-fourths of them the underlying cause was not coded as neurofibromatosis. The age distribution showed that NF1-associated deaths also occurred among the elderly, though mortality in early ages was high. The mean age for NF1-associated death was approximately 20 years lower than that for the general population. The gender differential may suggest that women are affected by more severe NF1-related complications, or they may simply reflect a greater tendency for NF1 to be reported on the death certificates of young women. Regarding the relation with other medical conditions, we found an excess, as the underlying cause of death, for malignant neoplasm of connective and other soft tissue and brain, but not for other sites. We also found an excess for obstructive chronic bronchitis and musculoskeletal system diseases among elderly persons.ConclusionThis is the first nationally representative population-based study on NF1-associated mortality in Italy. It stresses the importance of the Multiple-Causes-of-Death Database in providing a more complete picture of mortality for conditions that are frequently not recorded as the underlying cause of death, or to study complex chronic diseases or diseases that have no specific International Classification of Diseases code, such as NF1. It also highlights the usefulness of already available data when a surveillance system is not fully operational.

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Section: Discussionmentioning

confidence: 99%

Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

Masocco

Kodra²,

Vichi

et al. 2011

Orphanet J Rare Dis

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“…Langman et al [ 5 ] reported two cases of primary localized pleural neurofibroma out of which one was a known case of neurofibromatosis type I. Krishnamurthy et al [ 6 ], reported another case of isolated pleural neurofibroma in a 39-year-old female who presented with worsening chest pain. Another case of a 31-year-old male with pleural neurofibroma was reported by Gupta et al [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Primary Pleural Neurofibroma

Sharma¹,

Saxena²,

Seenivasagam³

et al. 2021

Cureus

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Metastatic deposits are the most common cause of pleural masses, solitary or multiple. Primary pleural neoplasms are rare entities that are occasionally encountered. Of these, the tumors of neurogenic origin are exceedingly rare with only a few cases reported in the literature. We describe a case of an isolated neurofibroma involving the pleura in a 23-year-old male patient, who underwent CT and MRI scans of the thorax as part of the initial investigations. The final diagnosis was clinched by a CT-guided biopsy of the lesion.

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“…Pleural involvement by neurofibroma is exceedingly rare with only three cases reported so far [12, 13]. Of these, only one case reported by Langman et al, was not associated with neurofibromatosis [13]. Primary pleural neurofibromas arise directly from intercostal nerves and form pleural based masses.…”

Section: Discussionmentioning

confidence: 99%

A Case of Isolated Primary Pleural Neurofibroma in a 39-Year-Old Woman

Krishnamurthy

Alexis

Basra

et al. 2019

Case Reports in Pulmonology

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Primary benign neurogenic neoplasms of the pleura are exceedingly rare. Neurofibromas rarely involve the pleura. A review of the literarture reveals only a single reported case of isolated pleural neurofibroma. Herein the authors describe another case of isolated primary pleural neurofibroma. A 39-year-old nonsmoker woman presented to the emergency room with complaints of progressively worsening chest pain of one month duration. A computed tomography of the chest revealed a crescent shaped, pleural based mass suspicious for a neurogenic tumor such as an intercostal schwannoma. A PET-CT skull base to midthigh failed to reveal any other masses or abnormalities. A surgical excision of the mass was performed due to the patient's intractable pain. The resected specimen consisted of an ovoid fragment of soft tissue with pale yellow, smooth and glistening cut surface. Microscopic examination revealed the tumor to be composed of spindle cells with wavy nuclei arranged haphazardly in loose collagenous and pale myxoid stroma with rare interspersed mast cells. The spindle cells were diffusely positive for S100 protein and SOX-10, and focally positive for neurofilament. In the absence of any other masses in the patient and no pertinent history, a diagnosis of primary pleural neurofibroma was made. This case emphasizes the need to consider neurofibroma in any spindle cell neoplasm of the pleura irrespective of age or singularity.

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Primary localised pleural neurofibroma: expanding the spectrum of spindle cell tumours of the pleura

Cited by 9 publications

References 13 publications

Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

A Rare Case of Primary Pleural Neurofibroma

A Case of Isolated Primary Pleural Neurofibroma in a 39-Year-Old Woman

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