Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

Muwakkit, Samar; Rodríguez‐Galindo, Carlos; Samra, Ahmad; Khoury, Ruby; Akel, Samir; Mroueh, Salman; Razzouk, Bassem I.; Abboud, Miguel R.

doi:10.1002/pbc.20787

Cited by 9 publications

(6 citation statements)

References 23 publications

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“…Our patient had a tumor huge in size compared to those of previous reports with multiple pleural nodules suggesting metastasis at diagnosis, which indicates delayed diagnosis despite her NF1 stigmata. It is well known that MPNST associated with NF1 shows a poor response to chemotherapy or radiotherapy and the prognosis with residual tumor or metastasis is dismal [1, 2, 18]. Although doxorubicin/ifosfamide- (AI-) based chemotherapy is known to be somewhat effective for adult MPNST and some pediatric cases [4, 23, 24], there is no known standard chemotherapy regimen for treatment of MPNST.…”

Section: Discussionmentioning

confidence: 99%

Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

Yoon

Lee

Chun

et al. 2014

Case Reports in Pediatrics

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Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue malignancy usually found in patients with neurofibromatosis type 1 (NF1) with a poor outcome. Although MPNST can be found in any part of the body including head and neck or extremities, intrathoracic MPNST with or without NF1 is uncommon, especially in children or adolescents. Reported herein is a case of huge intrathoracic MPNST in a 16-year-old girl with NF1, and a brief review of the literature.

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Section: Discussionmentioning

confidence: 99%

Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

Yoon

Lee

Chun

et al. 2014

Case Reports in Pediatrics

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“…Differential diagnosis of the solitary ground-glass nodule included primary lung cancer and an intrapulmonary neurinoma that could be related to neurofibromatosis [2]. …”

Section: Case Presentationmentioning

confidence: 99%

Lung Cancer Associated with Neurofibromatosis Type I

Oikonomou

Mikroulis

Mintzopoulou

et al. 2013

Case Reports in Radiology

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Lung cancer associated with neurofibromatosis type I is considered very rare, and only a few case reports have been described in the literature. There is some evidence that a genetic linkage between neurofibromatosis and carcinogenesis in the lung may exist. We present a 42-year-old female, lifetime nonsmoker with a known history of neurofibromatosis type I, free of respiratory symptoms, who underwent a low-dose HRCT of the lungs to investigate any occult interstitial lung changes. A solitary ill-defined nodule of a ground-glass opacity was detected incidentally in the middle lobe with no associated lymphadenopathy or metastatic disease. Several thin-walled lung cysts were also seen in the lower lobes. Histological analysis of the nodule after middle lobectomy revealed well-differentiated adenocarcinoma. The patient did not receive systemic chemotherapy or radiotherapy. She was free of disease on 18-month followup.

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“…8 There are also reports of various rare sarcoma entities such as synovial sarcoma and rhabdomyosarcoma, malignant peripheral nerve sheath tumors, and lymphoma with primary localization in the lung. [9][10][11][12][13][14][15][16] Inflammatory myofibroblastic tumors (IMT) assemble from inflammatory cells, collagen, and myofibroblastic mesenchymal spindle cells. 17,18 Due to their potential for local invasion and recurrence, the IMT were reclassified as intermediary lesions of borderline malignancy in 2015.…”

Section: Introductionmentioning

confidence: 99%

Incidences and characteristics of primary lung malignancies in childhood in Germany: An analysis of population‐based data from German cancer registries

Abele

Voggel

Bremensdorfer

et al. 2022

Pediatric Blood & Cancer

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Background: Primary lung malignancies are a heterogeneous group of cancers that occur very rarely in childhood. Due to limited knowledge of their epidemiologic and clinical features, these tumors present a challenge to the treating physicians. This study aimed to increase the knowledge about the occurrence of primary lung malignancies in childhood in Germany. Materials and methods: Pseudonymized data of cases recorded at the German Centerfor Cancer Registry Data (ZfKD) between 1990 and 2017 were retrieved. Primary lung malignancies were identified using the ICD-and ICD-O classification. Numbers were compared to those reported to the German Childhood Cancer Registry (GCCR). Crude incidence rates were calculated using the ZfKD database.Results: A total of 168 patients diagnosed with primary lung malignancies in the age below 19 years were identified from the ZfKD. The median age at diagnosis was 13 years. The most common tumor entities were lung carcinoids (n = 49), lung carcinoma (n = 36), and pleuropulmonary blastoma (n = 14). An unexpected accumulation of lung cancer cases was noted in the first year of life without a clearly specified histopathological diagnosis. A substantial discrepancy in the numbers of primary lung malignancies between ZfKD and GCCR was found. Conclusions:We present population-based data on the occurrence of primary childhood lung malignancies in Germany, which were more frequent than previously anticipated but likely remained underreported. For better understanding and optimal treatment of these entities, cancer registration needs to be improved through mandatory

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Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

Cited by 9 publications

References 23 publications

Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

Lung Cancer Associated with Neurofibromatosis Type I

Incidences and characteristics of primary lung malignancies in childhood in Germany: An analysis of population‐based data from German cancer registries

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