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Revista Española de Cirugía Oral y Maxilofacial

versión On-line ISSN 2173-9161versión impresa ISSN 1130-0558

Rev Esp Cirug Oral y Maxilofac vol.28 no.6 Madrid nov./dic. 2006




What should the diagnosis and treatment be?

¿Cuál es su diagnóstico y tratamiento?



A cervicofacial computed tomography (CT scan) was carried out with axial and coronal cuts that revealed a mass measuring 3 cm from the major axis to the posterior leftsided ethmoidal cells with extension to the nasal fossa and destruction of the medial orbital wall and medial wall of the maxillary sinus, with invasion of the left orbit and maxillary sinus. Infiltration of the lamina papyracea, sphenoid sinuses and orbital roof was not observed. The left eyeball was displaced forwards and sideways. The extrinsic eye muscles compared with the medial rectus muscle and interior rectus muscle were displaced, but there was no clear evidence of any infiltration. The mass was in intimate contact with the nasal septum but it had not extended over the midline (Fig. 2). As the likely diagnosis was of a tumor mass, two punch biopsies were carried out through the left nasal fossa using nasal forceps measuring 0.5 and 0.7 cm respectively. Both fragments had a whitish translucent color and a polypoid appearance. The microscopic examination revealed mucous lined by flat or polystratified epithelium that had diffuse neoplastic infiltration by the chorion of monomorphic cells that were round with scant cytoplasm and basophilic nuclei and "salt and pepper" chromatin pattern. There were some mitotic figures and abundant karyorrhexis. Occasionally nuclear molding and areas of necrosis could be observed (Fig. 3). The immunohistochemical study showed tumor cellularity with a positive phenotype for neuroendocrine markers (chromogranins, synaptophysin, enolase) and epithelial markers (Cam-5.2, Ck18), and negativity for S- 100, CD45-LC and enolase.


Small cell neuroendocrine carcinoma of the sinonasal region

Carcinoma neuroendocrino de células pequeñas de la región nasosinusal



R. González-García1, S. Hyun Nam-Cha2, F.J. Rodríguez Campo3, L. Naval Gías3, V. Escorial Hernández1,
P.L. Martos1, M. Mancha de la Plata1, F.J. Díaz González4

1 Médico Residente. Servicio de Cirugía Oral y Maxilofacial.
2 Médico Residente. Servicio de Anatomía Patológica.
3 Médico Adjunto. Servicio de Cirugía Oral y Maxilofacial.
4 Jefe de Servicio. Servicio de Cirugía Oral y Maxilofacial.
Hospital Universitario La Princesa. Madrid, España.

Dirección para correspondencia



The mass was diagnosed as small cell neuroendocrine carcinoma (poorly differentiated neuroendocrine carcinoma) of the left nasal fossa and left paranasal sinus. An extension study by means of a thoracic-abdominal CT scan was carried out that showed changes compatible with pulmonary fibrosis and mediastinal adenopathy, some of which were calcified, but without changes when compared with previous CT scans. Multiple cavitated lesions were also observed in both lungs, with moderately thick walls and, as a result of the aforementioned neoplasm, the existence of metastasis could not be ruled out, making the infectious etiology less probable. Small sclerotic lesions were also revealed in the pelvic girdle indicating possible metastasis given the antecedents of a primary tumor in the nasal fossa and paranasal sinuses.

Following evaluation by the Tumor Committee, it was decided that the initial treatment should consist of chemotherapy with three cycles of cisplatin and etoposide. Three months after starting the therapy, carrying out salvage surgery of the primary tumor was ruled out, given the persistence of the tumor mass, which was showing no significant changes in size, and the presence of nodules in both lungs with a lower pre-sternum location that had a post-biopsy histological diagnosis of metastasis of neuroendocrine carcinoma.



Small cell neuroendocrine carcinoma (SCNC) of the nasal or paranasal region is extremely rare. In fact, it constitutes the least prevalent tumor within sinonasal carcinomas with neuroendocrine differentiation (SCND). Its description as a clinicopathologic entity of the paranasal area was described by Chowdhuri 1 in 1965. In 1972, Oloffson and Van Nostrand 2 reported the first case in the larynx insisting in its histologic identity with small cell carcinoma of the lung. Other extrapulmonary locations have also been described including the skin, pancreas and cervix,3-5 with an incidence rate of around 4%, very much lower than the rates described for the lung.

Its origin has been attributed to the neuroendocrine system of APUD cells that are widely distributed across the organism. 6 This entity is histologically indistinguishable from SCNC of the lung. Both are solid tumors with small, regular sized cells that are organized in sheets, nests and cords. Many contain cribiform nuclei with a fine, reticular, chromatinic pattern and a moderate quantity of cytoplasm. In both tumors the presence of electron-dense neurosecretory granules adhered to the membrane is confirmed during the examination by electron microscopy.7 The immunohistochemical study is important in the differential diagnosis with other malignant entities, and it includes lymphoma, rhabdomyosarcoma, undifferentiated nasopharyngeal carcinoma and undifferentiated sinonasal carcinoma. Strongly positive staining for synaptophysin and CD56 (NCAM) can be observed and weak staining for chromogranin A and CAM 5.2/AE-1.8

Currently there is some controversy regarding the clinical behavior displayed by SCNC and its capacity for distant metastasis. Some authors9 have separated SCND into two macroscopic categories according to different clinical behavior, that is: esthesioneuroblastomas (ENB) and nonesthesioneuroblastoma, which includes undifferentiated sinonasal carcinoma, neuroendocrine carcinoma and small cell neuroendocrine carcinoma (SCNC). The first have excellent prognoses with regard to survival, local and distant control, and local therapy alone is carried out consisting in craniofacial resection and radiotherapy (the local control rate is: 86-96%). However, tumors with non-ENB histologic characteristics have poor survival rates, which could be related to the larger failure rates or recurrence rates at a systemic level. Various series affirm that SCNC represents the histological type with the greatest local and systemic recurrence rate.9,10 However, in spite of its histological identity with SMNC of the lung, some authors7 have described behavior development that is completely different in both locations, with regional or distant dissemination of nasal or paranasal SCNC being very rare. In these cases death occurs because of failure to control the disease locally, and as a result of aggressive behavior with a recurrence tendency.

While there is very little experience in the characterization of its clinical behavior, there is also little experience in how these types of tumors should be dealt with, although its histological similarity with SCNC of the lung has led to chemotherapy being considered the first modality of treatment. In fact Rosenthal et al. 9 have put the high rate of intracranial metastasis on the same level as that found in SCNC of the lung and, as a result, they recommend using systemic treatment based on chemo- and radiotherapy (with prophylactic cranial irradiation). According to this therapeutic approach, and after evaluation by the Tumor Committee, this case received initial treatment consisting in three cycles of cisplatin and etoposide given the possibility of lung metastasis as per the diagnosis. Soussi et al.11 also endorsed this therapeutic attitude. However, based on the different behavior reported by other authors, wide complete resection of the lesion would appear reasonable as initial treatment, with complementary radiotherapy and/or chemotherapy whenever local control appears to be a priority for this type of location. In this sense some authors 7report good results in terms of long-term survival when initial surgical therapy is carried out that is aggressive. This precept should only be applied in those cases in which the presence of metastasis at diagnosis can be ruled out.

To conclude, SCNC is a tumor entity that very rarely appears in the nasal fossa and paranasal sinuses, and there is little experience in how it should be handled. We believe that initial surgical treatment with complementary radioand chemotherapy is the best therapeutic option, although the use of neoadjuvant chemotherapy with cytoreduction followed by surgery and radiotherapy could be a valid alternative. In any event, given the high rate of local recurrence, a close long-term follow-up is necessary.



Dirección para correspondencia:
Raúl González García
c/ Los Yébenes 35, 8º C
28047 Madrid, España




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