Primitive neuroectodermal tumor of lungs in adults: a rare series of three cases treated with upfront chemo-radiation

Abstract

Primitive neuroectodermal tumors (PNETs) are highly malignant small round blue cell tumors of neuroectodermal origin belonging to either central nervous system, autonomic nervous system or peripheral Askin's or Ewing's group of neoplasms. The latter generally arise in soft tissues of trunk or axial skeleton in children and early adolescents. However in adults this entity is very uncommon. Of all peripheral entities, primary PNET of lungs without chest wall or pleural involvement in adults are extremely rare and have been scarcely reported in world literature as single case reports. We hereby report a series of three interesting cases of adult PNET of lung diagnosed and treated in our institute. The chief presenting complaints of these patients were of chest pain, cough and dyspnea. The cases were diagnosed on the basis of imaging and biopsy which confirmed these lesions to be of PNET histology, confirmed by immunopositivity for neuron specific enolase (NSE), synaptophysin, chromogranin, CD 99 and vimentin on immunohistochemistry (IHC). All three were deemed unresectable in view of infiltration of nearby vital organs and high chances of morbidity. They were treated with upfront chemotherapy followed by conformal radiotherapy (RT) to the residual disease to which they showed significant response both clinically and radiologically. Presently these patients are on regular follow-up for over 6 months without any evidence of progression of disease or distant metastasis.

Successful Radical Pneumonectomy for a Primitive Neuroendodermal Tumor in the Lung: A Case Report and Review of the Literature

“…Peripheral PNETs and Ewing's sarcoma belong to the Ewing family of tumors and are small round-cell malignancies originating from spinal cord cells and accounting for 5% of all small round-cell malignant neoplasms (11). Until now, only few pulmonary PNETs have been reported in the literature (12)(13)(14). Common clinical manifestations include chest pain, cough, and fever.…”

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

“…Metastatic disease involving lung, lymph nodes, and several organs has been described 12 , 48 , 49 ; however, primary ESFTs of breast, 50 - 52 lung, 53 - 55 intestine, 56 , 57 and brain 22 , 58 - 62 were rarely reported.…”

“…Further ESFTs in bladder and prostate noted in this cohort were rarely reported. [42][43][44][45][46][47] Metastatic disease involving lung, lymph nodes, and several organs has been described 12,48,49 ; however, primary ESFTs of breast, [50][51][52] lung, [53][54][55] intestine, 56,57 and brain 22,[58][59][60][61][62] were rarely reported.…”

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

“…Radiotherapy as local treatment approach is used when complete local resection is not feasible with a functional organ, a difficult anatomic location, or with very large tumor volume not amenable to radical surgery even after neoadjuvant chemotherapy, and in case of a metastatic disease. Post-operative radiotherapy has been implied to decrease local recurrence and provide prolonged survival 25,26 . The selection of local treatment modality is considerably biased by several factors, including tumor location, tumor volume, sensitivity to chemotherapy, patient general status, and institutional protocol.…”