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J Thorac Cardiovasc Surg 1994;107:960-962
© 1994 Mosby, Inc.
LETTERS TO THE EDITOR |
General Surgery Services
University Clinic Hospital Lozano Blesa of Zaragoza
Zaragoza, Spain
To the Editor:
In 1979, Askin and coworkers
1 described a unique clinical-pathologic condition characterized by the presence of a small-cell tumor in the thoracopulmonary region in 20 white children and adolescents (average age, 14
years) This is a rare disease in the pediatric agegroup, and even more rare in adults. Few adult cases have been described (we found only three cases in the literature). We report here one further case in an adult.
A 39-year-old white man who reported a history of morning cough from his smoking habit of 20 cigarettes a day for the previous 13 years and a duodenal ulcer came to the hospital, reporting an acute, continuous pain on the right subscapular area, radiating anteriorly. He had no cough, dyspnea, or fever. There was a leukocyte count of 12,500 cells/mm 3 with a normal differential count.
A chest radiograph showed an extrapulmonary mass close to the right thoracic wall with well-defined limits (Fig. 1). The suggested diagnoses were costal tumor and pleural effusion; the latter was disproved byechocardiography. A thoracic computed tomographic scan confirmed an extrapulmonary mass without costal participation or enlarged lymph nodes (Fig. 2). A fine-needle aspiration puncture was performed and the samples were analyzed according to Papanicolau techniques and Giemsa, noting the presence of a small-cell tumor with frequent mitoticimages. This set forth two possible diagnoses: sarcomatous and neurogenic tumors.
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1 included this tumor within the group of neurogenic tumors of the thorax, along with schwannomas, tumors from the sympathetic ganglia(ganglioneuromas and neuroblastomas), and paraganglia (pheochromocytoma). It is included as a peripheral neuroectodermic tumor by Shields and Reynolds 2 and as a malignant neuroepithelioma by Sternberg and colleagues. 3
Typically, Askin's tumor develops as a solitary mass or multiple masses in the thoracic area (thoracic wall, lung, mediastinum, or pericardium). Pain is the only or main symptom in 60% of cases. Seventy-five percent of Askin's tumors have been reported in white female patients younger than 20 years. 1 Rarely, Askin's tumors are found in the central nervous system. In the thoracic area, these tumors are invasive and prone to destroying bone (ribs and scapula), invading the retroperitoneal space, and spreading to lymph nodes, adrenals, and liver. Once resected, they recur with extremely high frequency. The most common recurrence sites are the skeleton, the sympathetic chain, and the original site.
Image diagnosis is based on plain chest radiographs, computed tomographic scan, and magnetic resonance imaging, the latter having the greatest sensitivity. Results of 1231-Tyr-3-octeotride scintigraphy are positive for most tumors and metastases. Fine-needle aspiration puncture followed by electron microscopic study identifies the presence of small cells with neurosecretory granules, microtubules, and cytoplasmatic extensions. 4 Unlike autonomic nervous system tumors, Askin's tumor does not produce biologically active substances, and catecholamine levels in urine are always within normal limits.
Conventional histologic features show compact nests of small cells, formation of Homer-Wright pseudorosettes with an acidophilic core of neurofibrillar characteristics, and infiltration of surrounding tissues. Neuron-specific enolase stain is considered as a good marker for this tumor and supports its neurogenic origin. 5 A reciprocal translocation t(11;22) (q24;12) has been demonstrated in Askin's tumor and Ewing's sarcoma. 6 This could indicate a common origin for these tumors.
The treatment for Askin's tumor must consist, whenever possible, of radical resection supplemented with radiotherapy (3500 to 5500 rad) and aggressive chemotherapy (cyclophosphamide, vincristine,doxorubicin, cysplatinum, methotrexate, bleomycin, and dimethyltriazenyl imidazole carboxamide). The average life expectancy is 8 months, although occasional long-term survival as long as 96 months has been reported.
The present case is unusual for the age of presentation and the short duration of the unique symptom, thoracic pain, which had started only hours before hospital admission (the reported mean duration of symptoms before diagnosis in these tumors is 6 months). Plain chest radiographs and computed tomographic scan were diagnostic for extrapleural thoracic wall tumor of nonosseous origin and fine-needle aspiration puncture was diagnostic for a small-cell tumor, with a high suspicion of malignancy. Altogether, the diagnostic workup led to what is currently considered to be correct initial treatment, completed before the histologic diagnosis was obtained, with the aggressive combined therapy this kind of tumor requires. Local infiltration was limited, an unusual feature in Askin's tumors. This may be related to the early diagnosis and prompt treatment.
We conclude that Askin's tumor should be considered as one etiologic possibility in a small-cell tumor of the thoracic wall at any age. Patients with such tumors consequently should be treated bymeans of prompt and wide local excision.
References
This article has been cited by other articles:
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  F. J. Baumgartner, B. O. Omari, and S. W. French Primitive neuroectodermal tumor of the pulmonary hilum in an adult Ann. Thorac. Surg., July 1, 2001; 72(1): 285 - 287. [Abstract] [Full Text] [PDF]
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