|Year : 2015 | Volume
| Issue : 4 | Page : 553-556
Extraosseous Ewing's tumor of larynx: A rare presentation
Vinod Shinde, Swapnil Gosavi, Rashmi Prashant, Devendra Jain, Gundappa D Mahajan, Nayanna Karodpati
Department of Ear, Nose and Throat, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. D.Y. Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Web Publication||14-Jul-2015|
304, Shreyas Apartments, Plot No. c-1, Sector 23, Nerul, Navi Mumbai - 400 706, Maharashtra
Source of Support: None, Conflict of Interest: None
Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-year-old male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation.
Keywords: Ewing family of tumors, larynx, primitive neural crest, primitive neuroectodermal tumor
|How to cite this article:|
Shinde V, Gosavi S, Prashant R, Jain D, Mahajan GD, Karodpati N. Extraosseous Ewing's tumor of larynx: A rare presentation. Med J DY Patil Univ 2015;8:553-6
|How to cite this URL:|
Shinde V, Gosavi S, Prashant R, Jain D, Mahajan GD, Karodpati N. Extraosseous Ewing's tumor of larynx: A rare presentation. Med J DY Patil Univ [serial online] 2015 [cited 2020 Aug 4];8:553-6. Available from: http://www.mjdrdypu.org/text.asp?2015/8/4/553/160821
| Introduction|| |
Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that originates from the cells of the primitive neural crest. PNET mainly occurs in the brain, extremities, pelvis, and the chest wall. The peripheral PNET (pPNET) is part of the Ewing's sarcoma family of tumor, which includes Ewing's tumor of bone, extraosseus Ewing tumor (tumor growing outside of the bone), PNET, and Askin tumor (PNET of the chest wall). Approximately, 9% arise in the upper aerodigestive tract or head and neck region, making it the third most common anatomic site, after the extremities and the thoracic/abdominal region.  Patients are typically children or adolescents. Here, we present a case study of PNET presenting in larynx in a 41-year-old male.
| Case Report|| |
A 41-year-old male presented with progressively increasing hoarseness for 3 months. He also had difficulty in breathing on exertion for 3 months and repeated episodes of stridor. On flexible nasopharyngoscopy, a smooth globular mass was seen in the subglottic region on the left side just below left vocal cord. [Figure 1] computed tomography (CT) scan showed a moderately large soft tissue lesion in the subglottic region at level of cricoid cartilage on the left side, not involving the cartilage, superiorly extending up to left vocal cord, highly suggestive of the neoplastic process. Magnetic resonance imaging neck [Figure 2]a-c] showed altered signal intensity mass arising from subglottic region measuring 1.7 cm (anteroposterior) to 1.5 cm (transverse) to 1.6 cm (× in a vertical axis) and extending superiorly up to the vocal cords. Borderline enlarged lymph nodes were seen at level IA and level II bilaterally.
|Figure 1: A smooth globular mass was seen in the subglottic region on the left side just below left vocal cord|
Click here to view
Tracheostomy was performed to secure the air way. Findings were confirmed with direct laryngoscopy, which showed a tumor in the subglottic region on the left side just below the left vocal cord and a pedunculated mass arising from the growth. The tumor was smooth, globular and biopsy from deeper tissues was taken and specimen sent for histopathological examination suggestive of small round cell tumor. Histopathology revealed a submucosal tumor composed of small cells having round nuclei, dispersed chromatin and scanty cytoplasm which appeared clear at places. The overlying squamous mucosa was normal [Figure 3]. Immunohistochemistry (IHC) was advised, which gave a diagnosis of PNET. The tumor was strongly positive for mic-2 [Figure 4] and vimentin immunostains. C-kit was expressed weakly. It was negative for chromogranin, synaptophysin, cytokeratin (CK), S-100 protein, myeloperoxidase (MPO), CD34, desmin and p63 immunostains. Immunohistochemistry ruled out small round cell tumor differential diagnoses of rhabdomyosarcoma (desmin negative), small cell carcinoma (CK, synaptophysin and chromogranin negative), basaloid squamous cell carcinoma (CK and p63 negative) and extramedullary round cell tumor (MPO and CD34 negative). Patient was screened for bone, abdomen and pelvic metastasis.
Patient was subsequently posted for microlaryngeal surgery, and the tumor was excised. Postoperatively, Patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide and mesna, along with granulocyte colony stimulating factor, with 21 days interval. Clinically the primary growth and lymph nodes regressed completely at the end of the therapy. After chemotherapy repeat CT scan, showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months postcompletion of all treatment. He is under regular follow-up and serial endoscopic evaluation at monthly intervals.
| Discussion|| |
Primitive neuroectodermal tumors are a group of highly malignant tumors composed of small round cells of neuroectodermal origin.
Primitive neuroectodermal tumor family of tumors have been classified into the following three groups based on the tissue of origin. 
Primitive neuroectodermal tumors and EFTs are referred as synonyms in the literature. Ewing sarcoma, however, is more common in bone, while PNETs are more common in soft tissues. Immuno-histochemical and cytogenetic studies suggest that all these tumors have a common origin. First described PNETs in 1918, these tumors were earlier thought to arise directly from nerves.  Based on molecular cytogenetic analysis, both Ewing's sarcoma and PNETs are known to share the same reciprocal translocations, most commonly between chromosomes 11 and 22. Further advances in immuno-histochemical analyses have helped further distinguishing PNETs and Ewing's sarcoma from other small, round, poorly differentiated tumors, including rhabdomyosarcoma, neuroblastoma, and lymphoma. PNETs often exhibit aggressive clinical behavior, with worse outcomes than other small, round cell tumors. Most pPNETs manifest in the thoracopulmonary region (Askin tumor), pelvis, abdomen, and extremities.  Rud et al. in a large series of 42 cases of extraosseous Ewing's sarcoma reported few cases in head and neck region.  In a series of 26 cases, Jones and McGill reported 11 of 26 patients with disease in the head and neck. Most other large published series, however, reveal a paucity of cases in the head and neck. ,, Of the published cases involving the head and neck, the sites of presentation are diverse, including, but not limited to, the paranasal sinuses, jugular foramen, oral cavity, nasal cavity, neck, skull, lingual nerve, parotid gland, larynx, retropharyngeal space, masseter, temporal area, pterygomaxillary space, and orbit.
- Central nervous system (CNS) PNETs - Tumors derived from the CNS.
- Neuroblastoma - Tumors derived from the autonomic nervous system.
- pPNETs - Tumors derived from tissues outside the central and autonomic nervous system. pPNETs are also classified as part of the Ewing family of tumors (EFTs).
Current recommendations include neoadjuvant chemotherapy with surgical excision or radiotherapy followed by adjuvant chemotherapy. Significant advances in the neoadjuvant and adjuvant chemotherapeutic regimens, as well as improved facility in diagnosing these tumors through cytogenetic and immuno-histochemical analysis, should improve long-term disease-free survival. 
| Conclusion|| |
Primitive neuroectodermal tumor is an aggressive malignant small round cell tumor that very rarely present in the head and neck. The involvement in the subglottic region is rarer and hence timely diagnosis, and treatment is a professional challenge. Long-term survival for patients with PNET is still poor; however, the use of newer chemotherapeutic agents combined with aggressive surgical control of primary disease and in some cases, radiation therapy should lead to improved long-term survival. 
| References|| |
Stelow EB. The larynx. In: Mills SE, Carter D, Greenson JK, Reuter VE, Stoler MH, editors. Sternberg's Diagnostic Surgical Pathology. 5 th
ed., Vol. 1. Philadephia: Wolters Kluwer Health and Lippincott Williams & Wilkins; 2009. p. 903-27.
Rick A, Brian M, Mary D, Bruce R. Extra skeletal Ewing's sarcoma. Cancer 1999;85:725-31.
Rud NP, Reiman HM, Pritchard DJ, Frassica FJ, Smithson WA. Extraosseous Ewing's sarcoma. A study of 42 cases. Cancer 1989;64:1548-53.
Jones JE, McGill T. Peripheral primitive neuroectodermal tumors of the head and neck. Arch Otolaryngol Head Neck Surg 1995;121:1392-5.
Kinsella TJ, Triche TJ, Dickman PS, Costa J, Tepper JE, Glaubiger D. Extraskeletal Ewing's sarcoma: Results of combined modality treatment. J Clin Oncol 1983;1:489-95.
Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, et al.
Ewing's sarcoma of soft tissues in childhood: A report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 1997;15:574-82.
Gururangan S, Marina NM, Luo X, Parham DM, Tzen CY, Greenwald CA, et al.
Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing's tumor with Ewing's-directed therapy. J Pediatr Hematol Oncol 1998;20:55-61.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]