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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 655-656
Alveolar rhabdomyosarcoma of mediastinum - a case report


Prof of Pathology, Govt Chest & General Hospital Hyderabad 500 038, India

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Date of Submission07-Jun-2005
Date of Acceptance08-Aug-2006
 

Keywords: Computed Tomography (CT), High-resolution Lung CT, Tuberculosis pulmonary

How to cite this article:
Moorthy N, Prasad B T, Sakunthala P, Bhramaramba K. Alveolar rhabdomyosarcoma of mediastinum - a case report. Indian J Radiol Imaging 2006;16:655-6

How to cite this URL:
Moorthy N, Prasad B T, Sakunthala P, Bhramaramba K. Alveolar rhabdomyosarcoma of mediastinum - a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Jan 18];16:655-6. Available from: http://www.ijri.org/text.asp?2006/16/4/655/32293
Alveolar rhabdomyosarcoma is an aggressive soft tissue malignancy of children and adolescents. It is extremely rare in mediastinum. We report a case of alveolar rhabdomyosarcoma of anterior mediastinum in a young male.

A 22 year old male patient presented with pyrexia, non productive cough with loss of appetite for the last three months. Since one month he developed constant chest pain and cough with foul smelling sputum. He had wheeze for the last twenty days.

Clinical examination revealed dull note with crepitations in right mammary region. No previous history of tuberculosis, diabetus,hypertension, asthma or airway disease.Routine lab tests were unremarkable. Elisa test was non reactive. Sputum for Gram stain showed cocci in pairs clusters. Chest radiograph showed a well defined anterior mediastinal mass without any calcifications. Rest of the lung parenchyma, heart and costophrenic angles

were normal.On ultrasonography a well defined large hypoechoic mass was seen in anterior hemithorax on right side. There was no plural effusion. Contrast enhanced CT scan chest showed a large heterogeneously enhancing soft tissue attenuation mass of size 9.7 x 11 cms seen in the anterior mediastinum. Fine calcification seen with in the mass.

There was segmental air space consolidation of upper lobe of right lung. The chest wall and pleura are invaded. There was no pleural effusion. The left lung and heart were normal.Ultrasound guided FNAC showed blood elements,lymphocytes, individually placed large cells, scant cytoplasm, hyperchromatic nuclei suggestive of germ cell tumor.

At surgery a large nodular whitish mass extending from the apex of lung to the diaphragm was seen blending inseparably with mediastinum, surface of the lung compressing the superior vena cava, inferior vena cava and heart. Debulking was done as the mass was inoperable. Biopsy revealed cells in alveolar pattern separated by fibrous septa. Cells are loosely arranged ,small, round with a hyperchromatic vesicular nuclei and prominent nucleoli. Cytoplasm is scanty. The features are in favour of a malignant small round cell neoplasm, alveolar rhabdomyosarcoma. Diastase degradable glycogen deposits were demonstrable by periodic acid-Schiff reagent.


   Discussion Top


Anterior mediastinum is the most common site of tumors in the mediastinum in general. Thymic tumors, germ cell neoplasms, lymphomas form the majority of the masses in the anterior mediastinum. Other malignant tumors that occur occasionally include liposarcoma, angiosarcoma, hemangiopericytoma, leiomyosarcoma, mesenchymal tumors etc. A few cases of granulocytic sarcoma, alveolar soft part sarcoma, synovial sarcoma were also reported.

Thymoma, the commonest primary neoplasm of the anterior mediastinum occur in middle age group [1]. They appear as round or oval homogenous soft tissue mass with well defined margins,typically situated in the region of the thymus. Focal calcifications may be seen with in the mass. The signs of invasion include complete obliteration of fat planes, pericardial thickening, encasement of mediastinal vessels, irregular interface with adjacent lung and focal or diffuse pleural thickening [2]. The thymic carcinoma usually present as large anterior mediastinal mass that has lobulated or poorly defined margins.

The mass may have homogenous or heterogenous attenuation with foci of calcification.

Obliteration of fat planes with invasion of pleura and pericardium seen in majority of cases. Thymic carcinoid tumor also appear on CT as homo or heterogeneously attenuated

mass with invasion of adjacent structures and metastases to regional lymphnodes and lung are not uncommon [2]. The skeletal metastases are osteoblastic. Non seminomatous malignant germ cell tumor appear as large anterior mediastinal mass with smooth, lobulated or irregular margins. They appear on CT with heterogenous attenuation and contain large areas of low attenuation. Focal calcification may be seen. Fat planes between the tumor and adjacent structures are usually obliterated. Extension of the mass into lung, chest wall and mediastinal metastases may be seen. Teratomas are well defined smooth masses with heterogenous attenuation due to its varied componenets.

Combination of fat , fluid and soft tissue attenuation suggests the diagnosis of cystic teratoma. Seminomas typically present as large masses with homogenous attenuation on CT with slight enhancement with contrast. Calcification occasionally may be seen.Invasion of adjacent structures is rare. Endodermal sinus tumor appear in young males and present with signs of mediastinal compression and systemic symptoms.

Hodgkin's disease is the most common lymphoma to affect the mediastinum and it presents with signs of respiratory distress or superior vena cava syndrome. Also reported in the mediastinum include synovial sarcoma, granulocytic sarcoma, pleomorphic adenoma etc.

Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence, usually arise in the deep muscles of the limbs. It is sub classified into

embryonal, alveolar and pleomorphic variants. Histologially the alveolar subgroup is traversed by a network of fibrous septae that divide the cells into clusters. As the central cells degenerate and drop out a crude resemblance to pulmonary alveolae is created [3]. Rhabdomyosarcoma is exteremely rare in mediastinum. Suster [4] reported four cases of primary anterior mediastinal tumors in young adults that were characterized by solid, infiltrative lesions showing histologic and immunohistochemical features of

rhabdomyoblastic differentiation. Solid alveolar rhabdomyosarcoma of thorax was described in a10 year boy[5]. Atypical biological behavior of alveolar rhabdomyosarcoma in 5 patients was discussed by Duran Padilla MA et al [6].

PAX3/FKHR fusion transcript, a genetic marker is specific for alveolar rhabdomyosarcoma. Immunohistochemical positive staining with vimentin, desmin, titin and antibodies was suggestive of rhabdomyosarcoma. Rhabdomyosarcoma is an aggressive tumor and treated with surgery and chemotherapy and the prognosis is poor.

 
   References Top

1.H. Page McAdams, Jeremy J.Eramus, Robert D et al mediastinum chap 29 in.John R. Haaga, Charles F. Lanzieri, Robert C. Gilkeson eds CT and MR Imaging of the Whole Body vol one 4th ed Missouri v:Mosby 2003 : 937-996  Back to cited text no. 1    
2.Chapter 74: Masses situated predominantly in the anterior mediastinal compartment in R.S.Fraser, Neil Cocman, Nester L.Muller in Fraser and Pare's Diagnosi of disease of the chest vol 4 4th ed Philadelphia W.B Sanders 1999 p2875-2912  Back to cited text no. 2    
3.Chapter 26 : Bones, joints and soft tissue tumors in. Vinay kumar Abul K.Abbas, Nelson Fausto Robbins and Cotran Pathologic basis of disease Philadelphia W.B.Sanders.  Back to cited text no. 3    
4.Suster S, Moran CA, Koss MN rhabdomyosarcomas of the anterior mediastinum: report of 4 cases in Hum Pathol 1994 apr; 25(4): 349-56.  Back to cited text no. 4    
5.Sartelet H, Lantuejoul S, Armari-Alla C, Pin I, Delattre O, Brambilla E solid alveolar rhabdomyosarcoma of the thorax in a child Histopathology. 1998 feb ; 32(2): 165-71  Back to cited text no. 5    
6.Duran Padilla MA, Rodriguez Martinez HA, Chavez Mercado L, Soto Abraham M V et al . atypical biological behavior of alveolar rhabdomyosarcoma in 5 patients Rev Invest Clin 1999 jan-feb 51(1) 17-22   Back to cited text no. 6    

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Correspondence Address:
NLN Moorthy
Prof of Radiology, Govt chest & General Hospital, 3-4-526/39 flat no 9 Aditya apartments barkatpura Hyderabad 500027
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32293

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