Primary dedifferentiated liposarcoma of the lung with rhabdomyoblastic and chrondroblastic differentiation

Longano, Anthony; DuGuesclin, Alexandra; Mitchell, Catherine

doi:10.1111/his.12410

Cited by 7 publications

(8 citation statements)

References 8 publications

(34 reference statements)

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“…[5] Exhaustive literature search suggests that fewer than twenty cases have so far been reported. [1,2] It shows a slight male predilection as seen in our case too with reported patient age range of 9-59 years. [5,6] The presenting symptoms simulate that of lung tumor such as dry cough or with expectoration, hemoptysis, chest pain, fever, dyspnea, reduced appetite and general weakness, so on and so forth.…”

Section: Discussionsupporting

confidence: 69%

“…Primary pulmonary liposarcoma is an extremely rare malignancy with <20 cases reported in literature. [1,2] Intrathoracic liposarcomas are uncommon, and if at all encountered, are usually located in the mediastinum. [3] Malignant degeneration of a pulmonary lipoma and pleuropulmonary asbestosis have been considered as possible pathogenetic factors.…”

Section: Introductionmentioning

confidence: 99%

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Primary Pulmonary Liposarcoma with Pancreatic Metastasis: A Rarest of Rare Intrathoracic Malignancy

Halder

Biswas

Shukla

et al. 2020

Indian Journal of Medical and Paediatric Oncology

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Liposarcoma of the lung is an extremely rare malignancy with <20 cases reported so far. With such limited evidence, natural history and appropriate management of this unusual tumor is unclear. We experienced a case of primary pulmonary myxoid liposarcoma in a 34-year-old male who presented with multiple distant site metastases including pancreas, which is again an extremely rare finding accounting for approximately 2% of pancreatic malignancies. Indexed case was treated with systemic chemotherapy.

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Section: Discussionsupporting

confidence: 69%

Section: Introductionmentioning

confidence: 99%

Primary Pulmonary Liposarcoma with Pancreatic Metastasis: A Rarest of Rare Intrathoracic Malignancy

Halder

Biswas

Shukla

et al. 2020

Indian Journal of Medical and Paediatric Oncology

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“…There have been numerous reports of DDLPS with rhabdomyosarcomatous differentiation. 7,8,[31][32][33][34][35] The rhabdomyoblastic component can resemble any type of rhabdomyosarcoma; however, in one study a pleomorphic rhabdomyosarcoma pattern was observed in 50% of cases of DDLPS with rhabdomyosarcomatous differentiation. 8 Also, only 2 of 20 cases of DDLPS had a round cell morphology similar to ERMS.…”

Section: Discussionmentioning

confidence: 99%

Molecular Characterization of a Rare Dedifferentiated Liposarcoma With Rhabdomyosarcomatous Differentiation in a 24 Year Old

et al. 2019

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Aims. The aim of this study was to identify potential driver genetic alterations in a dedifferentiated liposarcoma (DDLPS) with rhabdomyosarcomatous differentiation. Methods and Results. A 24-year-old female underwent resection of an abdominal mass, which on a previous biopsy demonstrated rhabdomyosarcomatous differentiation concerning for embryonal rhabdomyosarcoma. Histologically the resected tumor displayed a high-grade sarcoma with rhabdomyosarcomatous differentiation in the background of well-differentiated liposarcoma consistent with DDLPS. Fluorescence in situ hybridization confirmed MDM2 amplification, as did array-based copy number profiling. Whole-exome sequencing revealed a somatic FGFR1 hotspot mutation and RNA sequencing an LMNB2-MAP2K6 fusion only within the dedifferentiated component. Conclusions. This study represents an in-depth examination of a rare DDLPS with rhabdomyosarcomatous differentiation in a young individual. Additionally, it is also instructive of a potential pitfall when assessing for MDM2 amplification in small biopsies. Despite exhaustive analysis, mutation and gene copy number analysis did not identify any molecular events that would underlie the rhabdomyoblastic differentiation. Our understanding of what causes some tumors to dedifferentiate as well as undergo divergent differentiation is limited, and larger studies are needed.

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“…[4,15] Primary lung LS is very uncommon, most of the reported cases are myxoid, pleomorphic, and unclassified subtypes and less commonly dedifferentiated subtype. [21] In liposarcomas of the lung and mediastinum, it is important to differentiate them from pleural LS if there is chest wall or mediastinal invasion. This must be made clinically, with imaging evaluation or surgically [Figure 7].…”

Section: Head and Neck Liposarcomasmentioning

confidence: 99%

Liposarcoma: A Pictorial and Literature Review

Santoscoy¹,

Castillo²,

Jose³

et al. 2018

J Clin Res Radiol

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According to some clinical trials and epidemiologic studies, WDL is the most common LS (40%), followed by myxoid LS (20%), DDLS, pleomorphic LS (5%), and, last, mixed-type LS. [3,6,8] There are crucial differences among each subtype regarding presentation, treatment, and prognosis. WDL WDL is the most common subtype, representing the 40-50% of all LSs. WDL is a low-grade tumor due to their highly lipomatous content. Histologically, they are composed of variable size mature adipocytes, with scattered lipoblast and large fibrous stroma, and in some cases, they may present sclerosing and inflammatory components.

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Primary dedifferentiated liposarcoma of the lung with rhabdomyoblastic and chrondroblastic differentiation

Cited by 7 publications

References 8 publications

Primary Pulmonary Liposarcoma with Pancreatic Metastasis: A Rarest of Rare Intrathoracic Malignancy

Primary Pulmonary Liposarcoma with Pancreatic Metastasis: A Rarest of Rare Intrathoracic Malignancy

Molecular Characterization of a Rare Dedifferentiated Liposarcoma With Rhabdomyosarcomatous Differentiation in a 24 Year Old

Liposarcoma: A Pictorial and Literature Review

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