PÁGINA DEL RESIDENTE

What is your diagnosis and treatment plan?

¿Cuál es su diagnóstico y tratamiento?

The patient was male, 64 years old, and he had retired after working in a steel foundry for 42 years. He had a medical history of hepatic cysts that had not been treated surgically, intestinal polyps that were being followed by the Digestion department in our hospital, and COPD that was being treated with inhaled corticosteroids.

The Service of Otorhinolaryngology had referred him to our department because, for eight months, he had been suffering from recurrent epistaxis and nasal obstruction that was compatible initially with nasal polyps.

The examination of the patient revealed an excrescent mass in the left nasal fossa with a lobulated appearance that was brownish in color and friable in consistency. It blocked the lumen of the nasal fossa practically completely (Fig. 1). The cervicofacial examination was absolutely normal, and there were no adenopathies or associated lesions in the neck. The examination of organs and tracts was unremarkable.

A preoperative study was requested, together with imaging studies (Computerized Axial Tomography and Nuclear Magnetic Resonance) and a biopsy of the lesion.

The results of the preoperative study were normal, and no abnormalities were found in the blood tests, nor in the coagulation parameters, nor in the rest of the tests requested.

MRI (Fig. 2) showed a mass that was nearly completely occupying the left nasal fossa, and which was destructuring the whole area of the turbinates. The lesion was expanding towards the more anterior portion of the nasal fossa as well as towards the nasopharyngeal cavity. There was evidence of bulging of the nasal septum, and infiltration by the lesion could not be ruled out. There was partial destructuring of the more caudal portion of the ethmoidal cells*. No infiltration of the ethmoidal cribriform plate could be appreciated nor of the frontoethmoidal transition. Minimal distention was observed of the medial wall of the left orbit and of the floor of this orbit with no infiltration in the area.

The biopsy of the lesion revealed partially necrotic tissue with inflammatory cells together with cells with a malignant neoplastic appearance and a plasmocytoid tendency. There was positivity for HMB45, vimentin and S100. Ki-67 showed a proliferative index that was 40% positive.

Finally, a Positron Emission Tomography (PET) was requested that showed hypermetabolic foci in the more anterior portion of the nasal fossa as well as by the choanae. The rest of the examination did not reveal any other lesions.

After the diagnostic study of the lesion, surgical treatment was carried out.

Mucosal melanoma of the paranasal sinuses

Melanoma mucoso de senos paranasales

L.M. Capitán Cañadas¹, I.L. Labrot Moleón¹, D.J. Durán Moreno¹, A. Cabello Serrano¹, E. Valencia Laseca²

1 Médico Residente.
2 Jefe de Servicio.
Servicio de Cirugía Oral y Maxilofacial. Hospital Universitario Virgen de las Nieves. Granada, España

Correspondence

Based on the histopathologic diagnostic suspicion of melanoma, a tumorectomy was decided on (Fig. 3) that included a segmental hemimaxillectomy. This was carried out by means of a combined coronal/transfacial approach together with assisted endoscopic surgery for the complete resection of the mucosa of the ethmoidal sinus and the mucosa of both maxillary sinuses. For reconstructing the defect, a pedicled flap of galea-pericranium was decided on. The tumor excised consisted of a polypoid friable mass that measured 4x4x2.7cm in diameter. It was in various irregular fragments that were obtained using an endoscopic approach (Fig. 4).

The histopathologic study of the surgical specimen confirmed the diagnosis of epithelioid mucosal melanoma and of round cells with wide hemorrhaging and necrotic areas (Fig. 5). The polyps, as well as the mucosa of the ethmoid and maxillary sinuses, were disease-free.

The immediate postoperative period was satisfactory and complication-free.

The patient received postoperative tridimensional conformal radiotherapy to the surgical area. A dose of 30 Gy was used and a hypofractionated regimen with a dose of 3 Gy per fraction per day, five days a week, using 6 MV photon beam. The postoperative evaluation 12 months after the surgery showed the absence clinically and radiologically of any disease (Figs. 6 and 7).

Discussion

Mucosal melanoma of the paranasal sinuses (MMPNS) is considered extremely aggressive pathology¹ that represents approximately 1% of the total number of melanomas and between 3 and 4% of the total number of tumors of the paranasal sinuses.^2,3 The estimated incidence rate is 1 per 500.000 inhabitants per year.³ It tends to affect females and patients over the age of 60.⁴ In up to 80% of patients the disease appears in a localized area principally in the nasal cavity.² The main clinical symptoms related with MMPNS are epistaxis, the presence or sensation of a mass and obs his tructive symptoms.⁵ In addition there may be other minor symptoms associated with respiratory difficulties, congestion, polyps and pain.⁶ The appearance of melanorrhea and nasal secretions of a brown color, suggest that there is melanin pigment. ⁵ As opposed to cutaneous melanomas, the degeneration from precursor lesions is unusual. The main metastasic targets of MMPNS are the brain and lungs,⁷ and the presence of local neck metastasis is infrequent. Around a third of these lesions present with little or no pigmentation, which makes their diagnosis more difficult.⁸

The diagnostic management of MMPNS includes carrying out imaging studies, principally CAT scans and MRI, and systemic tumor screening that can be supported by PET studies and histopathologic tumor filiation.³

On a histological level there may be certain diagnostic doubt with regard to poorly differentiated carcinomas, lymphomas, plasmacytomas, rhabdomyosarcomas and neuroblastomas of the olfactory tract.⁵ The immunohistochemical profile of MMPNS is identical to that of cutaneous melanomas, as there are positive reactions for S100 protein, tyrosinase and HMB45.⁹

In spite of a great variety of staging systems having been proposed for melanomas in general, there is no uniformity with regard to the classification of MMPNS. This is due to the difficulty in applying to MMPNS the main staging criteria that have been proposed by different authors for cutaneous melanoma (identification of the histological limits such as papillary or reticular dermis, tumor thickness). The recent work by Thompson et al.⁵ supported by the 115 patients with MMPNS in their study, proposes a staging system based on the classical TNM system with certain modifications that permit managing these lesions easily.

There is no doubt that the treatment of choice for MMPNS entails surgical excision of the tumor.^2,3,5,10 Despite the open approach being the most frequently used,¹¹ the use of endoscopic surgery has been advocated in order to facilitate excision in areas with difficult access, or as a primary approach for tumors that have previously been selected.¹² As the rate of neck node metastasis is not above 5%, carrying out systemic neck depletion is not recommended. ² Although there is consensus for applying postoperative radiotherapy,¹³ numerous publications affirm that this will not increase the survival rates of MMPNS patients.⁵ A recent publication on the issue by Temam et al.¹⁴ suggests that there is increased local control of the disease after applying postoperative radiotherapy. The efficiency of using chemotherapy in patients with MMPNS has not been demonstrated, neither when combined with radiotherapy nor with surgical treatment nor when used alone, and its use is limited to advanced stages with systemic tumor invasion. ¹³In spite of immunotherapy having been used for treating certain types of cutaneous melanomas,¹⁵ randomized trials are necessary to show the effectiveness of this type of therapy in patients with MMPNS.³

Local recurrence is the principal factor associated with therapeutic failure. The appearance of recurrence is associated principally with incomplete excision of the tumor as a result of the complex and irregular anatomical relationships in the paranasal region, and with the presence of multifocal tumors, submucosal tumor proliferation and tumor seeding during surgery.⁵

The prognosis generally for MMPNS is poor and associated principally with tumor size, a delay in the diagnosis as a result of the unspecific nature of the symptoms, and because removing the tumor completely is not possible as a result of it being inaccessible technically.⁵ The survival rate at five years for patients with MMPNS varies between 20 and 40%.^2,5

Conclusions

MMPNS are extremely aggressive tumors, and the survival rate is under 40%. The treatment of choice for this tumor is surgical excision. Complete resection and tumorfree margins is the only demonstrated means of achieving adequate control of the disease. While there is no evidence as to improved survival, most authors advocate the administration of postoperative radiotherapy.

Correspondence:
Luis Miguel Capitán Cañadas
Servicio de Cirugía Oral y Maxilofacial
Hospital Universitario Virgen de las Nieves CRT.
Carretera de Jaén s/n Granada, España
E-mail: luismxf@hotmail.com

References

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