Askin tumor in the chest wall.

Askin's tumor is a kind of aggressive tumor and extremely rare. To date, the treatment is uncodified and the prognosis for patients with this tumor remains bleak. Herein we report a case of a giant Askin's tumor in the chest wall that following diagnosis was successfully treated with surgical resection.

Askin's tumor is a kind of aggressive tumor and extremely rare. To date, the treatment is uncodified and the prognosis for patients with this condition remains bleak. Herein we report a case of a giant Askin's tumor in the chest wall that following diagnosis was successfully treated with surgical resection. A 31‐year‐old male patient was referred to our center following an incidental radiological finding of a mass in the chest wall. A computed tomography (CT) scan of the chest revealed a 13 × 8.3 cm mass with an unclear boundary enclosing the lung in the left chest wall (Fig 1a–c). Both his past and family history were uneventful. Preoperative examinations were all within normal limits. Following multidisciplinary discussion, the patient underwent thoracotomy for resection of the mass. En bloc resection with reconstruction of the chest wall using titanium mesh and lobectomy of the left upper lobe with negative macroscopical and microscopical resection margins was successfully performed (Fig 1d and f). Postoperative histopathology, cytological examination and molecular analysis confirmed the mass was an Askin's tumor (Fig 1e). The patient subsequently received four cycles of chemotherapy with epirubicin and dacarbazine, and no significant recurrence was observed two years after surgery (Fig 1f).

Figure 1

(a–c) Chest computed tomography (CT) scan showed a mass (red arrow) and the surrounding structures. (d) En bloc resection of the tumor. (e) Hematoxylin and eosin (H&E) staining showed the tumor was comprised of small round blue cells (200× manifestation). (f) CT scan showed no significant recurrence two years after surgery.

Primitive neuroectodermal tumor (PNET) including primitive neuroectodermal central nervous system tumor, neuroblastoma and peripheral primitive neuroectodermal tumor belongs to Ewing's sarcoma family of tumors which is a kind of aggressive tumor. Among them, peripheral PNET in the chest wall (Askin's tumor) is extremely rare, and was first reported by Askin et al. in 1979. It often occurs in children and adolescents, but can develop at any age, with a female predominance in about 75% of cases. There may be systemic symptoms such as breathlessness, cough, and weight loss. Patients usually present with an asymptomatic mass on computed tomography (CT) scan, with or without pain. To date, it remains a challenge to surgeons due to the absence of standard therapeutic guidelines on the diagnosis and treatment of Askin's tumor, leading to a poor prognosis with two‐ and six‐year survival rates of 38% and 14%, respectively. Despite the poor prognosis, treatment of Askin's tumor requires discussion by multidisciplinary tumor working groups. Aggressive treatment include surgery, chemotherapy, and radiation should be undertaken, and complete surgical resection is recommended for a definitive diagnosis and individual treatment.

Disclosure

None declared.