International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 3 , Pages 180-184, September 2007

A peripheral primitive neuroectodermal tumor arising from the middle turbinate and transnasal endoscopic approach for its surgical treatment

  • Mete Iseri

      Affiliations

    • Kocaeli University Medical Faculty, Department of Otorhinolaryngology, Kocaeli, Turkey
    • ,
  • Murat Ozturk

      Affiliations

    • Kocaeli University Medical Faculty, Department of Otorhinolaryngology, Kocaeli, Turkey
    • Corresponding Author InformationCorresponding author at: Kocaeli Universitesi Tip Fakultesi, KBB Anabilim Dali, 41380 Umuttepe, Kocaeli, Turkey. Tel.: +90 262 3038872; fax: +90 262 3037003.
    • ,
  • Deniz Filinte

      Affiliations

    • Kocaeli University Medical Faculty, Department of Pathology, Kocaeli, Turkey
    • ,
  • Funda Corapcıoglu

      Affiliations

    • Kocaeli University Medical Faculty, Department of Pediatry, Kocaeli, Turkey

Received 8 March 2007; received in revised form 15 May 2007; accepted 15 May 2007.

Article Outline

Summary 

Malignant peripheral primitive neuroectodermal tumors (pPNETs) account for very little in all sarcomas. Sinonasal involvement is encountered even very rarely. In this case report, a very uncommon growth of pPNET in a 10-year-old girl arising from the lateral side of the middle turbinate in the sinonasal region was described and its treatment by transnasal endoscopic approach and chemotherapy was discussed.

Keywords: Primitive neuroectodermal tumor, Middle turbinate, Sinonasal region

 

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1. Introduction 

Peripheral PNET (pPNET) family of tumors is rare malignancies accounting for 1% of all sarcomas [1]. These tumors are classified in small round cell tumors which are composed of undifferentiated cells often lack mature phenotypic features, and are difficult to distinguish from each other and to classify. The pPNET term is describing the group of tumors presumed neural crest origin that presented outside the central and sympathetic nervous systems. These tumors are rare in the head and neck, the most common involvement site is the orbit, where as sinonasal involvement is very rare [2].

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2. Case report 

A 10-year-old girl was referred to our otorhinolaryngology department with the complaint of epistaxis from the right nasal cavity. On physical examination, a polypoid mass in the right nasal cavity was realized and a punch biopsy was done. The histopathologic examination showed concordant findings with a PNET family of tumors. She had moderate right nasal obstruction for about 1 year. Endoscopic examination showed a polypoid rough reddish mass obstructing completely the right nasal cavity. The patient had no other complaints, and the physical examination was otherwise normal. Maxillofacial magnetic resonance scans demonstrated a 25mm×22mm mass lesion giving hyperintense signal on the right nasal cavity (Fig. 1). There was suspicion of the right orbit medial wall invasion on the MRI, but the paranasal sinus CT showed the right orbit medial wall as intact (Fig. 2). The thorax CT, abdominal ultrasonography and the cranial MRI were normal. By the radiographic findings, an endoscopic approach to remove the tumor was found suitable. After a preoperative preparation, the planned endoscopic surgery was done. Intraoperatively, this polypoid mass was damaging the middle turbinate and pushing it medially, and was obstructing the entire right nasal passage (Fig. 3). The mass was originating from the insertion of the middle turbinate to the skull base (Fig. 4). It was not extending to the anterior skull base and there was no invasion of lamina papyracea. The tumor was removed and frozen sections were done to be sure of excising it completely. The surgical margins were free of tumor. After a nasal packing, the operation was completed. No complications were seen postoperatively. On histopathologic examination, the tumor cells were round, with oval nucleus and large eosinophilic cytoplasm, and were forming cordons and groups (Fig. 5). Atypical mitoses were seen frequently. Immunohistochemical studies demonstrated positive immunoreactivity for neurone-spesific enolase (NSE) (Fig. 6) and synaptophysin; weak immunoreactivity for S-100 protein; focal positive immunoreactivity for EMA (Fig. 7); and negative immunoreactivity for LCA, CD20, CD99, CD3, CD34, CD31, MPO, desmin, keratin and chromogranin. The diagnosis of malignant PNET were made with these histological and immunohistochemical findings. After this surgical therapy, the patient was referred to medical oncology and a chemotherapy protocol including vincristine 1.5mg/m2 1 day, adriablastina 20mg/m2 3 days, and iphosphamide 2g/m2 3 days was administered. After 8 months follow-up, the patient was free of disease, and there were no signs of metastasis or recurrence.

  • View full-size image.
  • Fig. 3. 

    Endoscopic view of the right nasal cavity. The polypoid mass is damaging the middle turbinate, and pushing it medially (S: septum; MT: middle turbinate; IT: inferior turbinate; tm: tumor).

  • View full-size image.
  • Fig. 4. 

    On the endoscopic operation, after the debulking of the tumor, the origin of the mass was found as the insertion point of the middle turbinate to the skull base (S: septum; tm: tumor; *the insertion point).

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3. Discussion 

PNETs are poorly differentiated small round cell tumors of common neuroectodermal origin. The term of central PNET describes the tumors arising from the central nervous system, such as medulloblastoma. The tumors which are arising from the autonomic nervous system are called neuroblastoma as a PNET group. Peripheral PNET is the other group outside of aforementioned groups describing tumors arising outside the central and autonomic nervous systems [3]. The commonest location of these malignancies is the thoracopulmonary region, about the rate of 46% [4]. The frequency of pPNET in head and neck region varies among different studies. In large series, 23 or 42% of the tumors represented in the head and neck, but in other series the rates are more rare [2]. Because of the rarity and the difficulties in pathologic diagnosis, these tumors’ incidence is not clear.

When the tumor involves the head and neck region, after the orbit, the nasal cavity with or without paranasal sinuses is the most common side of tumor growth as a primary lesion [5]. It can also arise from the neck, the oral cavity, the infratemporal fossa, the skull base, the larynx, parotid gland, mandible and gingiva [5]. pPNET can develop at any age, but most of the patients are less than 20-year old [5]. A rapidly enlarging, often painful mass is the most frequent clinical presentation, but cranial nerve deficits can also be seen. Metastases are possible at the initial presentation. Our patient's symptoms were unilateral nasal obstruction and epistaxis. Thus, unilateral nasal obstruction in a child should be carefully evaluated in term of potential malignancies. As we saw in the endoscopy, these tumors are appearing as fragile, destructive, bleeding nasal masses. They have an irregular wall structure with reddish appearing.

Preoperative CT and MRI are essential for the evaluation of the tumor margins and anatomic relations. In addition, the planning of the treatment modality especially the optimal surgical approach will require these radiographic studies. While CT usually shows a heterogeneous mass, it is isointense on T1 and hyperintense on T2-weighted MRI scans. Proton MR spectroscopy can also use in depicting metastasis and spreading to surrounding tissues, and provides useful information in clinical differentiation between PNETs and common brain tumors [6], [7]. In this case, the radiological findings gave us the idea of endoscopic approach to remove the tumor, in stead of external approaches. The reasons for this decision were: (1) no invasion to the skull base; (2) no invasion to the periorbital space; (3) no invasion to the lateral nasal wall; (4) being a stalked polypoid tumor arising from the middle turbinate attachment point.

Small, round, blue cells are common morphologic character of many tumors, such as PNET, rhabdomyosarcoma, lymphoma, Ewing's sarcoma, etc. The presence of rosette formation, perhaps, is the most helpful histological future, but lots of malignant pPNETs do not have it just like our patient. The pathologic diagnosis depends on exclusion of similar neoplasms and demonstration of neural differentiation which can be varying degrees. Positive immunostaining for at least two neural markers such as neurone-spesific enolase (NSE), synaptophysin, chromogranin, neurofilament, S-100, etc. is essential. NSE which is being positive in most of patients, synaptophysin and S-100 were positive immune neural markers having role in diagnosis of our patient. In addition, the atypical mitoses were also showing the malignant character.

The differential diagnosis includes a various types of tumor such as malignant lymphoma, malignant melanoma, embryonal rhabdomyosarcoma, poorly differentiated salivary gland tumors, undifferentiated nasopharyngeal carcinoma, olfactory neuroblastoma and the Ewing's sarcoma family of tumors.

When a pPNET kind of tumor is encountered, a multidisciplinary treatment protocol should be planned. Their malignant and progressive character needs aggressive surgical resection if it is possible. Chemotherapy or radiotherapy should also be combined with; to treat residual disease and to prevent from recurrence. There still exists no standard protocol for the pPNET family of tumors. A multiagent chemotherapy including vincristine, doxorubicin, and cyclophosphamide has been suggested for residual disease, and it may be helpful for treating undetected or detected metastasis or recurrent disease [5]. For patients who have unresectable tumor or who do not want surgery, radiotherapy is the remaining alternative modality, but it is not too useful alone. The preferred surgical approach for the sinonasal tumors is craniofacial resection. Endoscopic surgery had been used usually to get tissue for histopathologic examination or to tumor ablation [2]. But in this case, the radiologic and endoscopic findings were showing a limited tumor in the nasal cavity. If pPNET family of tumors is diagnosed early enough, it can be successfully treated with endoscopic surgery obtaining adequate tumor free margins.

These tumors are known as very aggressive and have tendency to recurrence and metastasize. Survival is generally poor; the major prognostic factors can be described as tumor site, tumor volume, and the presence of metastasis. In addition endocranially extended tumors show a rapidly growth and lethal outcome. Akeyson et al found a mean survival time of approximately 12 months [8]. In general, survival rates for 2 and 3 years are about 65 and 56% [5]. Because of the high recurrence rates, patients must be followed-up closely to realize as early as tumor recurrence and also possible improvement of metastasis.

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4. Conclusion 

In this case, the follow-up period may be a little less. The going on follow-up will show us the real success of our endoscopic approach this particular case. More cases will make us more informed, but a pPNET in the nasal cavity is a very rare condition, therefore it is difficult to say the most suitable surgical way. If we diagnose these tumors earlier with no advanced invasion to the surrounding tissues, it may be possible to excise them endoscopically in stead of external approaches.

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References 

  1. Enzinger FM, Weiss SW. Primitive neuroectodermal tumors and related lesions. In:  Enzinger FM,  Weiss SW editor. Soft Tissue Tumors. 3rd ed.. St. Louis: Mosby-Year Book; 1995;p. 929–964
  2. Nikitakis NG, Salama AR, O’Malley BW, Ord RA, Papadimitriou JC. Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature. Head Neck. 2003;25:488–498
  3. Batsakis JG, Mackay B, el-Naggar AK. Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report. Ann. Otol. Rhinol. Laryngol. 1996;105:838–843
  4. Alobid I, Bernal-Sprekelsen M, Alos L, Benitez P, Traserra J, Mullol J. Peripheral primitive neuroectodermal tumour of the left maxillary sinus. Acta Otolaryngol. 2003;123:776–778
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  6. Syal R, Reddy SJ, Kumar R, Tyagi I, Abrar AA, Krishnani N, et al. Unusual clinical and MRI features of a cerebellopontine angle medulloepithelioma. Case report and review of literature. Pediatr. Neurosurg. 2006;42:299–303
  7. Kovanlikaya A, Panigrahy A, Krieger MD, Gonzalez-Gomez I, Ghugre N, McComb JG, et al. Untreated pediatric primitive neuroectodermal tumor in vivo: quantitation of taurine with MR spectroscopy. Radiology. 2005;236:1020–1025
  8. Akeyson EW, McCutcheon IE, Pershouse MA, Sleek PA, Fuller GN. Primitive neuroeciodermal tumor of the median nerve. Case report with eytogenetic analysis. J. Neurosurg. 1996;85:163–169

PII: S1871-4048(07)00043-3

doi:10.1016/j.pedex.2007.05.005

International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 3 , Pages 180-184, September 2007

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