Elsevier

World Neurosurgery

Volume 103, July 2017, Pages 953.e1-953.e5
World Neurosurgery

Case Report
Metastatic Alveolar Soft Part Sarcoma of the Spinal Cord: A Case Report and Review of Literature

https://doi.org/10.1016/j.wneu.2017.04.100Get rights and content

Background

Alveolar soft part sarcoma (ASPS) is a rare, malignant soft-tissue neoplasm typically seen in young adults that possesses an unusual tendency to metastasize. Metastases to the intramedullary compartment of the spinal cord, however, are exceptionally rare and have not been described in the literature.

Case Description

We report the case of a 23-year-old woman with disseminated ASPS to the lung and brain who presented with progressive lower-extremity weakness and loss of sensation after radiation and chemotherapy. Magnetic resonance imaging revealed a 1.3-cm avidly enhancing lesion within the central thoracic spinal cord at T3. A T2–T4 laminectomy was undertaken and resulted in a gross total resection. Histopathologically, the mass was composed of organoid nests containing epithelioid cells with eosinophilic, granular cytoplasm separated by sinusoidal spaces. Immunohistochemistry demonstrated convincing positive TFE3 staining. Postoperative imaging confirmed the complete resection of the mass, and her examination was notable for intact sensation and impaired motor function that gradually improved.

Conclusions

A review of the literature found that the reported case represents the first instance of primary or metastatic ASPS in the spinal cord. Metastatic ASPS should thus be included in the differential diagnosis in patients with known disease and neurologic impairment or back pain. Imaging of the spine should then be considered.

Introduction

Alveolar soft part sarcoma (ASPS) is a rare, malignant soft-tissue neoplasm that constitutes approximately 0.5%–1.0% of all soft-tissue sarcomas.1, 2 This tumor is uniquely characterized by a nonreciprocal chromosomal translocation, t(X;17)(p11.2;q25), that results in the formation of a novel fusion gene, ASPL-TFE3.3 In addition to its highly specific genetic makeup, ASPS also exhibits distinctive histologic features, which include an alveolar pattern of clustered polygonal cells separated by fibrovascular septae and the presence of diastase-resistant periodic acid-Schiff–positive crystalline cytoplasmic granules.4, 5 The most common presentation of ASPS is in the skeletal muscle and deep soft tissue of the leg or thigh; however, a majority of patients, estimated at 65% in one study, present with disseminated stage IV disease.6 Similar to other sarcomas, ASPS has a predisposition for metastasizing to lung and bone while also possessing an unusual propensity to metastasize to the brain.2, 7 Consequently, small series of patients with brain metastases have been described in the literature.6, 8, 9, 10 Interestingly, cases of patients with suspected primary intracranial manifestations of ASPS also have been reported.5, 9, 11, 12, 13, 14 Primary tumors and metastases also have been noted in close proximity to the spinal cord within the bony spinal column.15, 16, 17 However, to our knowledge, there are no reports of ASPS involvement of the spinal cord in the English literature. Here, we present a unique and unfortunate case of a 23-year-old woman with disseminated disease to the lung and brain who subsequently developed an intramedullary thoracic spinal cord metastatic focus of ASPS.

Section snippets

Clinical History

A 23-year-old woman presented with headaches and an enlarging painless mass in her distal right thigh that was detected approximately 1 year before consultation. Subsequent magnetic resonance imaging (MRI) of the head revealed multiple brain metastases, with the largest measuring 1.7 × 1.37 cm in the right occipital lobe (Figure 1A–C). These images did not demonstrate meningeal enhancement. An MRI of the right thigh confirmed the presence of a 17.9 × 6.3 × 7.6-cm mass, whereas computed

Discussion

ASPS is most prevalent in younger individuals, with a peak incidence between 15 and 35 years and a female predominance during the first 2 decades of life.6, 7, 18 On presentation, the primary tumor most commonly develops in the deep soft tissues of the lower extremities and trunk in adults, while generally originating in the head and neck region in children.7, 12, 16 In many cases, patients detect a soft, asymptomatic mass, which prompts them to seek medical care. Although localized ASPS often

Conclusions

We present a case of disseminated ASPS with a unique metastatic presentation, whereby a metastasis was observed in the intramedullary spinal cord with no surrounding bony lesions. This case may represent the first instance when ASPS has involved the spinal cord in any manner. Treatment with complete resection may have halted the progression of neurologic motor and sensory symptoms, even though long-term neurological improvement could not be assessed. Knowledge of this case could be useful when

Acknowledgements

All authors were responsible for the preparation, review, or approval of the manuscript, and all are included in the decision to submit the manuscript for publication.

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  • Cited by (2)

    • Molecular Landscape in Alveolar Soft Part Sarcoma: Implications for Molecular Targeted Therapy

      2018, Biomedicine and Pharmacotherapy
      Citation Excerpt :

      The number of ASPS recorded articles in PubMed has grown gradually over last 20 years (since 1997 to 2017) (Fig. 1). Unlike most sarcomas, ASPS has high tendency for metastasis [20]. This type of cancer has tendency toward distant metastasis and invasion especially to brain and lungs [21]; However uncommon sites of metastasis such as distal phalanx of hand has been reported [22].

    Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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