A rare case of pleuropulmonary synovial sarcoma of the chest wall: A case report and a literature review

Abdulghaffar, Shareefa; AlNuaimi, Dana; AlMulla, Muna; Eldoky, Yahia; Farhan, Rabia; Kumar, Navin; Khairi, Tarig

doi:10.1016/j.radcr.2020.11.008

Cited by 3 publications

(6 citation statements)

References 11 publications

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“…4,5 El SS es un tumor maligno de origen mesenquimático que, a pesar de su nombre, no está relacionado con los tejidos sinoviales. 8 Representa el 8 % de los sarcomas de partes blandas. Se localiza con mayor frecuencia en las extremidades, aunque también puede tener localización axial (cabeza, cuello, pared abdominal, retroperitoneo, mediastino o pleuropulmonar), la cual se asocia a un peor pronóstico y requiere tratamiento más intensivo, como en el caso de nuestro paciente.…”

Section: Discussionunclassified

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Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso

2023

Arch Argent Pediat

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El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años.Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada.Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo.

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Section: Discussionunclassified

“…6,7 Cuando se manifiesta de forma primaria en pulmón, el sarcoma sinovial pleuropulmonar (SSPP) presenta mal pronóstico y, en general, un curso agresivo de la enfermedad. 8 En ocasiones, los neumotórax presentan mala evolución con persistencia o recurrencia, 9,10 lo que marca el rumbo de realizar estudios en búsqueda de causa subyacente.…”

Section: Introductionunclassified

Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso

2023

Arch Argent Pediat

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“…SS is a malignant tumor of mesenchymal origin that, despite its name, is not related to synovial tissues. 8 It accounts for 8% of soft tissue sarcomas. SS is most frequently located in the limbs, although it may also have an axial location (head, neck, abdominal wall, retroperitoneum, mediastinum, or pleuropulmonary), which is associated with a worse prognosis and requires more intensive treatment, as in the case of our patient.…”

Section: Figure 2 High-resolution Computed Tomography Of the Chest No...mentioning

confidence: 99%

“…6 PPSS is an uncommon form of SS and accounts for 0.1-0.5% of lung tumors; the few cases described so far are aggressive and affect adolescents and young adults. 6,8 PPSS manifests most frequently as an asymptomatic lung mass or with chest pain, cough, dyspnea, and even hemothorax. 7 On chest X-ray, it is seen as a well-defined, rounded-edged lung or pleural mass usually accompanied by ipsilateral pleural effusion.…”

Section: Figure 2 High-resolution Computed Tomography Of the Chest No...mentioning

confidence: 99%

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Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

et al. 2023

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Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1-0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years.Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed.In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

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Primary pleural synovial sarcoma with diffuse pleural involvement: A case report

Mutevelizade

Sayit

2023

Médecine Nucléaire

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A rare case of pleuropulmonary synovial sarcoma of the chest wall: A case report and a literature review

Cited by 3 publications

References 11 publications

Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso

Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso

Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

Primary pleural synovial sarcoma with diffuse pleural involvement: A case report

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