open access

Vol 85, No 4 (2017)
CASE REPORTS
Published online: 2017-07-31
Submitted: 2017-02-18
Accepted: 2017-04-09
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Primary pleuropulmonary synovial sarcoma with brain metastases in a paediatric patient: an unusual presentation

Pushpak Chandrakant Chirmade, Sonia Parikh, Asha Anand, Harsha Panchal, Apurva Patel, Sandip Shah
DOI: 10.5603/ARM.2017.0034
·
Adv Respir Med 2017;85(4):206-210.

open access

Vol 85, No 4 (2017)
CASE REPORTS
Published online: 2017-07-31
Submitted: 2017-02-18
Accepted: 2017-04-09

Abstract

Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

Abstract

Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

Get Citation

Keywords

peadiatric sarcoma, primary pleuropulmonary synovial sarcoma; brain metastases, SS18 rearrangement, TLE positive

About this article
Title

Primary pleuropulmonary synovial sarcoma with brain metastases in a paediatric patient: an unusual presentation

Journal

Advances in Respiratory Medicine

Issue

Vol 85, No 4 (2017)

Pages

206-210

Published online

2017-07-31

DOI

10.5603/ARM.2017.0034

Bibliographic record

Adv Respir Med 2017;85(4):206-210.

Keywords

peadiatric sarcoma
primary pleuropulmonary synovial sarcoma
brain metastases
SS18 rearrangement
TLE positive

Authors

Pushpak Chandrakant Chirmade
Sonia Parikh
Asha Anand
Harsha Panchal
Apurva Patel
Sandip Shah

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