|LETTER TO EDITOR
|Year : 2011 | Volume
| Issue : 2 | Page : 269-270
Pleural rhabdomyosarcoma: A rare entity
N Chaudhary, A Borker
Department of Pediatrics, Kasturba Medical College, Manipal, Karnataka, India
|Date of Web Publication||11-Jul-2011|
Department of Pediatrics, Kasturba Medical College, Manipal, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chaudhary N, Borker A. Pleural rhabdomyosarcoma: A rare entity. Indian J Cancer 2011;48:269-70
Rhabdomyosarcoma (RMS) is an aggressive malignant tumor of childhood arising from striated muscle, commonly located in the head and neck region, genitourinary tract, and the extremities. Thoracic RMS is uncommon and pleural RMS is extremely rare with only eight cases reported in literature so far. 
A 13-years-old girl presented with fever, dry cough, left-sided chest pain, and exertional dyspnoea of one-week duration. Examination revealed tachypnea, tachycardia, silent and stony dull left hemi-thorax with mediastinal shift to right side.
Chest X-ray revealed massive left-sided pleural effusion. Computed tomography (CT) scan of the chest confirmed massive pleural effusion and multiple large heterogeneously enhancing pleural masses involving mediastinal and lateral pleura, left chest wall postero-laterally and left crus of the diaphragm [Figure 1]. Emergency thoracocentesis was performed and pleural fluid examination revealed exudative malignant effusion. CT-guided biopsy from the masses revealed embryonal RMS. The immunohistochemistry was positive for desmin and myogenin, focally positive for mic-2 and was negative for creatine kinase, S-100 and synaptophysin. Bone scan revealed multiple bone metastases involving manubrium sterni, right scapula, left 8th and 10th ribs, D9, D10, D11, and L5 vertebrae, left ischium and sacroiliac joint. CT scan of the abdomen and the pelvis was normal. Bone marrow biopsy was negative for malignancy.
|Figure 1: CT thorax showing multiple large heterogeneously enhancing pleural masses (black arrows) with massive pleural effusion on left side|
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Cyclical chemotherapy with vincristine, ifosfamide, etoposide, alternating with vincristine, doxorubicin, cyclophosphamide and vincristine, actinomycin-D, cyclophosphamide was started. Clinical and radiological evaluation at 9 weeks showed that she had complete resolution of the pleural masses and the pleural effusion with significant regression of the bony metastases. After 24 weeks of chemotherapy whole body positron emission tomography (PET) scan was negative for any abnormal uptake anywhere in the body. She received 14 Gray of radiotherapy to the primary site and 40 weeks of chemotherapy. The patient is alive and in remission 15 months after diagnosis.
Thoracic RMS is rare with a reported incidence of 3.8% in IRS I through III studies. , A case series of 15 patients with chest wall RMS, identified from a data base of 303 consecutive RMS cases (5%), has been published.  The pleura is one of the rarest site of RMS with only eight previously reported cases in literature. 
Chest wall tumors usually present with pain and swelling at local site. Tumors may occasionally remain asymptomatic till they reach large dimensions. Pleural effusion resulting in dyspnoea may lead to earlier diagnosis.
CT scan is the initial imaging modality to assess local extent of the tumor. Histopathological examination of mass lesion, if any, is essential for definitive diagnosis, but pleural fluid cytology with immunohistochemical staining may be used reliably in the absence of detectable mass lesion.  Thoracic and abdominal imaging, bone scan and bilateral bone marrow biopsy are integral parts of a staging work-up.
Thoracic RMS has a poorer prognosis due to larger size, advanced stage at presentation, unresectability and alveolar histology. Unresectability of the lesion and presence of multiple bony metastases were poor prognostic factors in our patient. Complete response documented by CT and PET at both primary and metastatic sites avoided the need for second look surgery. Incomplete response to chemotherapy may lead to aggressive attempts at delayed surgical resection, combined with appropriate post-operative radiotherapy. 
| » References|| |
|1.||Ayadi L, Chaabouni S, Chabchoub I, Ayadi A, Kallel R, Fakhfakh I, et al. Primary rhabdomyosarcoma of the pleura presenting as recurrent pneumothorax. Rev Mal Respir 2009;26:333-7. |
|2.||Raney RB, Maurer HM, Anderson JR, Andrassy RJ, Donaldson SS, Qualman SJ, et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): major lessons from the IRS-I through IRS-IV studies as background for the current IRS-V treatment protocols. Sarcoma 2001;5:9-15. |
|3.||Andrassy RJ, Wiener ES, Raney RB, Lawrence W, Lobe TE, Corpron CA, et al. Thoracic sarcomas in children. Ann Surg 1998;227:170-3. |
|4.||Saenz NC, Ghavimi F, Gerald W, Gollamudi S, LaQuaglia MP. Chest wall rhabdomyosarcoma. Cancer 1997;80:1513-7. |
|5.||Theunissen P, Cremers M, van der Meer S, Bot F, Bras J. Cytologic diagnosis of rhabdomyosarcoma in a child with a pleural effusion: A case report. Acta Cytol 2004;48:249-53. |