Alveolar rhabdomyosarcoma of gluteus maximus

Clinical Cases 30.04.2012
Scan Image
Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 27 years, male
Authors: Mahmood A
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Details
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AI Report

Clinical History

A 27-year-old man presented with a right buttock mass, and constitutional symptoms i.e. fever, fatigue and malaise for a few weeks.

Imaging Findings

Contrast enhanced CT chest, abdomen and pelvis was requested which showed a lobulated, noncalcified soft tissue attenuation mass arising from the gluteus maximus and extending to the skin surface (Fig. 1). No underlying bone destruction was seen. Similar soft tissue attenuation masses were seen in numerous skeletal muscles throughout chest and abdomen (Fig. 2-3). Multiple pulmonary nodules were seen consistent with metastases (Fig. 4). A necrotic metastatic node was present in the right groin (Fig. 1). On the basis of imaging, diagnosis of sarcoma with pulmonary, skeletal muscle and lymph node metastases was suggested.

Histological diagnosis was requested by oncologists for chemotherapy, therefore the right gluteal mass was core biopsied under ultrasound guidance, which confirmed the diagnosis of alveolar rhabdomyosarcoma.

MRI of the right gluteal mass was not performed because of widespread distant metastases. Unfortunately, the patient did not respond to chemotherapy, and was subsequently started on palliative care.

Discussion

Rhabdomyosarcoma (RMS) is the most common connective tissue tumour under 20 years of age [1]. It originates from undifferentiated mesenchymal cells.
Commonly, it is found in the head and neck region (44%) followed by paratesticular region and retroperitoneum (20.4%), and extremities (14.6%) [2].
Histologically, RMS can be classified into embryonal tumours, more common in young children and often seen in the urogenital tract, and alveolar rhabdomyosarcoma which tends to affect adolescents with a predilection for deep soft tissues of the extremities.
Alveolar subtype has a poor prognosis. Factors associated with poorer prognosis are adult onset of disease, alveolar subtype and tumour greater than 6 cm in diameter [3, 4].
Cross-sectional imaging, typically CT is essential to delineate the extent of locoregional and metastatic disease. Alveolar RMS typically appears as a soft tissue attenuation mass with some enhancement on CT, and echogenic or mixed density lesions on ultrasound. MRI of alveolar RMS shows a heterogenous mass, which indicates haemorrhage or necrosis. The mass appears isointense to muscle on T1W imaging and shows intensity equal to or greater than fat on T2W imaging. It also demonstrates considerable enhancement following administration of gadolinium.
The most common sites of metastases are lymph nodes, lungs and liver.
Franco et al tried to differentiate sub-types of rhabdomyosarcoma on the basis of imaging but were unsuccessful [5]. Orell et al mentioned that aspirations from these tumours are highly cellular with a consistent pattern of small cell tumour with predominantly single cells or stripped nuclei; cells are mainly uniform with rounded or irregular nuclei and scanty cytoplasm [6].
Treatment options depend upon the tumour subtype and stage, and include surgery, chemotherapy and radiotherapy. Due to poorer prognosis, chemotherapy is usually required in almost all patients. Excision of primary site is recommended if tumour is diagnosed at an early stage, however, local recurrence is common. Overall mortality depends upon the patient’s age and tumour site, with 5-year survival highest in children aged 1-4 years (77%) and worst in infants (47%) and adolescents (48%) [7].

Teaching points: Alveolar rhabdomyosarcoma can present as a subcutaneous mass in a young patient beyond the age of adolescence. Multimodality approach is essential for optimum patient care, and clinical radiology plays a central role in patient management.

Differential Diagnosis List

Alveolar rhabdomyosarcoma of gluteus maximus
Soft tissue metastases
Multiple abscesses

Final Diagnosis

Alveolar rhabdomyosarcoma of gluteus maximus

Liscense

Figures

Contrast enhanced axial CT pelvis

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Contrast enhanced axial CT pelvis

Contrast enhanced axial CT abdomen

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Contrast enhanced axial CT abdomen

Contrst enhanced axial CT chest

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Contrst enhanced axial CT chest

Contrast enhanced axial CT chest

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Contrast enhanced axial CT chest