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
·
Pubmed: 28871588
·
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.

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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

Pubmed

28871588

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

References (25)
  1. Keel SB, Bacha E, Mark EJ, et al. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol. 1999; 12(12): 1124–1131.
  2. Etienne-Mastroianni B, Falchero L, Chalabreysse L, et al. Primary sarcomas of the lung: a clinicopathologic study of 12 cases. Lung Cancer. 2002; 38(3): 283–289.
  3. Cohen MC, Kaschula RO. Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature. Pediatr Pulmonol. 1992; 14(4): 222–232.
  4. Hancock BJ, Di Lorenzo M, Youssef S, et al. Childhood primary pulmonary neoplasms. J Pediatr Surg. 1993; 28(9): 1133–1136.
  5. Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. J Surg Oncol. 2008; 97(4): 314–320.
  6. Sakellaridis N, Mahera H, Pomonis S. Hemangiopericytoma-like synovial sarcoma of the lumbar spine. Case report. J Neurosurg Spine. 2006; 4(2): 179–182.
  7. Krieg AH, Hefti F, Speth BM, et al. Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors. Ann Oncol. 2011; 22(2): 458–467.
  8. Greene S, Hawkins DS, Rutledge JC, et al. Pediatric intradural extramedullary synovial sarcoma: case report. Neurosurgery. 2006; 59(6): E1339; discussion E1339.
  9. McGrory JE, Pritchard DJ, Arndt CA, et al. Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience. Clin Orthop Relat Res. 2000(374): 247–258.
  10. Koss M, Travis W, Moran C. Pulmonary sarcomas, blastoma, carcinosarcomas and teratomas. In: Hasleton PS, Travis W, Moran C. ed. Spencer's pathology of the lung. 5th ed. McGraw Hill, New York 1996: 1065–1069.
  11. Collins BT, Janney CG, Ong M, et al. Fine needle aspiration biopsy of monophasic spindle synovial sarcoma of lung with fluorescence in situ hybridization identification of t(x;18) translocation: a case report. Acta Cytol. 2009; 53(1): 105–108.
  12. Zamarrón C, Abdulkader I, Alvarez UC, et al. Primary synovial sarcoma of the lung. Intern Med. 2006; 45(10): 679–683.
  13. Zeren H, Moran CA, Suster S, et al. Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. Hum Pathol. 1995; 26(5): 474–480.
  14. Hosono T, Hironaka M, Kobayashi A, et al. Primary pulmonary synovial sarcoma confirmed by molecular detection of SYT-SSX1 fusion gene transcripts: a case report and review of the literature. Jpn J Clin Oncol. 2005; 35(5): 274–279.
  15. Spurrell EL, Fisher C, Thomas JM, et al. Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital. Ann Oncol. 2005; 16(3): 437–444.
  16. Essary L, Vargas S, Fletcher C. Primary pleuropulmonary synovial sarcoma. Cancer. 2002; 94(2): 459–469.
  17. Jaques DP, Coit DG, Casper ES, et al. Hepatic metastases from soft-tissue sarcoma. Ann Surg. 1995; 221(4): 392–397.
  18. Bakri A, Shinagare AB, Krajewski KM, et al. Synovial sarcoma: imaging features of common and uncommon primary sites, metastatic patterns, and treatment response. AJR Am J Roentgenol. 2012; 199(2): W208–W215.
  19. Kaufman J, Tsukada Y. Synovial sarcoma with brain metastases. Report of a case responding to supervoltage irradiation and review of the literature. Cancer. 1976; 38(1): 96–99.
  20. Siegel HJ, Dunahm WH, Lopez-Ben R, et al. Intracranial metastasis from synovial sarcoma. Orthopedics. 2008; 31(4): 405–407.
  21. Boroumand N, Raja V, Jones DV, et al. SYT-SSX2 variant of primary pulmonary synovial sarcoma with focal expression of CD117 (c-Kit) protein and a poor clinical outcome. Arch Pathol Lab Med. 2003; 127(4): e201–e204.
  22. Nuwal P, Dixit R, Shah NS, et al. Primary monophasic synovial sarcoma lung with brain metastasis diagnosed on transthoracic FNAC: Report of a case with literature review. Lung India. 2012; 29(4): 384–387.
  23. Weiss SW, Goldblum JR. Enzinger's and Weiss's soft tissue tumors. 5th ed. . Mosby Elsevier, Philadelhia 2008: 1161–1182.
  24. Mikami Y, Nakajima M, Hashimoto H, et al. Primary poorly differentiated monophasic synovial sarcoma of the lung. A case report with immunohistochemical and genetic studies. Pathol Res Pract. 2003; 199(12): 827–833.
  25. Hirakawa N, Naka T, Yamamoto I, et al. Overexpression of bcl-2 protein in synovial sarcoma: a comparative study of other soft tissue spindle cell sarcomas and an additional analysis by fluorescence in situ hybridization. Hum Pathol. 1996; 27(10): 1060–1065.

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