gms | German Medical Science

ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge
7th Congress of the European Skull Base Society held in association with
the 13th Congress of the German Society of Skull Base Surgery

18. - 21.05.2005, Fulda, Germany

Malignant frontobasal melanoma – prognosis and impact of therapeutic strategy

Meeting Contribution

  • Wolfgang Maier - Department of Oto-Rhino-Laryngology, University Medical School and Clinics, Freiburg, Germany
  • Petra U. Lohnstein - Department of Oto-Rhino-Laryngology, University Medical School and Clinics, Freiburg, Germany
  • Thomas Klenzner - Department of Oto-Rhino-Laryngology, University of Düsseldorf, Düsseldorf, Germany
  • Joerg Schipper - Department of Oto-Rhino-Laryngology, University of Düsseldorf, Düsseldorf, Germany

ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge. 7th Congress of the European Skull Base Society held in association with the 13th Congress of the German Society of Skull Base Surgery. Fulda, 18.-21.05.2005. Düsseldorf: German Medical Science GMS Publishing House; 2009. Doc05esbs13

DOI: 10.3205/05esbs13, URN: urn:nbn:de:0183-05esbs134

Published: January 27, 2009

© 2009 Maier et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Introduction

Malignant melanoma of the rhinobasal mucosa is very rare and makes up less than one percent of all malignant melanomas [1]. The tumour is clinically silent for a long time. Symptoms are late and unspecific in most cases, and therefore at the time of diagnosis the patients´ tumour volume is often large. During the past two decades, several therapeutic options have been proposed like various forms of chemotherapy, interferon therapy, adjuvant or primary irradiation, and combinations of these therapeutic modalities [1], [2]. Therefore it seems to be important to evaluate the impact of surgical and noninvasive therapy strategies.

Methods

In a retrospective quality assessment, we analyzed the charts of all 11 consecutive patients suffering from malignant frontobasal melanoma who where treated in our department since 1993. We collected the following data: initial symptoms, tumour stage (according to the UICC 2002 classification for tumours of the inner nose, ethmoidal and maxillary sinuses), the region of initial tumour origin, histologic examination (melanotic, amelanotic), therapeutic strategy initially applied to the individual patient, disease free interval, survival time, and status at present. If recurrence occurred, we noted site and stage of recurrence, therapy and outcome. We evaluated the influence of specific surgical (radical-mutilating or functional) and non-surgical (radiotherapy, immunotherapy, chemotherapy) therapy on recurrence and outcome.

Results

Initial symptoms were unspecific in 9 of 11 patients, including nasal bleeding and obstruction. Thus, melanoma was an accidental finding of a biopsy (4 cases under suspicion of papilloma or carcinoma), conchotomy (3 cases) or sinus surgery (2 cases) in 9 patients, including all cases with amelanotic melanoma. In one 84 years old patient a black tumour was visible inside the nose, and this was the only case in which the presurgically correct suspicion diagnosis was histologically confirmed. A cervical metastasis without primary tumour (T0 N2a) was initial symptom in one patient, and despite thorough clinical and endoscopic examination the primary tumour (hard palate) was found only 2 years later. This primary tumour had obviously grown in the submucosa and arroded the bone of the hard palate. The primary tumour of the other 10 patients was in the inner nose or the sinuses. 3 of them had a T1 stage when diagnosis was made, 3 a T2 stage, one a T3, and 3 patients a T4a stage.

All patients underwent surgery as initial treatment. This was immediately followed by immunotherapy in 4 patients (interferon-alpha), by transcutaneous irradiation in 2 patients, or by chemotherapy in one patient. We did not find a dependance of the initial tumour stage and the patients´ survival (Figure 1 [Fig. 1]). When stratifying the patients according to the primary tumour´s stage, the survival time of 7 patients with no visible or a small tumour (T0, T1 or T2) was even shorter than that of 4 patients with a T3 or a T4a melanoma. Furthermore, there was no obviously different survival between 7 patients suffering from pigmentated melanoma compared to 4 patients with amelanotic melanoma (Figure 1 [Fig. 1]). We could not see a different outcome in 4 patients treated initally by surgery solely in comparison to the 7 patients receiving additional adjuvant radio-, immuno-, or chemotherapy. Neither, prognosis was obviously influenced by metastatic affection of cervival lymph nodes, nor by mucosal infiltration of the naspoharyngeal roof. However, as the number of cases was limited, a statistical evaluation could not be performed.

Initial surgery aimed at complete resection of the tumour with maintenance of integrity and function of vitally or functionally important structures like the eyes, or the internal carotid artery. This strategy of limited radicality achieved R0 resection in all but 2 patients who were treated in our department from the beginning. One of the patients in whom R0 resection was not achieved suffered from multiple nasal and pharyngeal melanosis, so a safe R0 resection of all melanotic fields was impossible. In this man early and repeated recurrence from multiple melanotic fields occurred, and he underwent a number of surgical interventions and irradiation. He died almost 5 years later from brain metastases. In the other patient, a 78 years old woman, a T4a melanoma of the sinuses, the nasopharynx, and the periorbita was diagnosed, and she had suffered from metastasizing mamma cancer before. To avoid orbital exenteration, she underwent R1 resection of the melanoma and consecutive irradiation. She died 6 months later from a thromboemboly of the lung without clinical evidence of residual melanoma. In one patient only, after melanoma recurrence in the ethmoid invading the orbita, mutilating surgery with exenteration of the orbit was performed. In spite of preceding chemotherapy and following interferon therapy, she died of multiple tumour spread few months later. Today, 5 of 11 patients are still alive, all of them clinically free from tumour. The longest survival was achieved in a woman who was 59 years old when diagnosis was made. She initially presented with a T4a melanoma and survived for 8 years and 5 months, in spite of multiple local and regionary recurrence which was treated with function-preserving surgery and adjuvant interferon therapy. She died from massive bleeding, probably carotid artery arrosion.

Discussion

When comparing our data with those published in the literature, we suppose that prognosis of malignant frontobasal melanoma has not developed favourably during the past two decades [1], [2]. The initial stage of the primary tumour, surprisingly, was not predictive for survival, nor was the presence or absence of regional lymph node metastases. Large tumour volume and local recurrence do not limit prognosis provided curative (but not mutilating) surgery is performed. In contrast to reports from the literature [2], we did not observe a worse prognosis in patients suffering from amelanotic compared to pigmentated melanoma, nor in cases with involvement of nasopharyngeal mucosa. As amelanotic melanoma can mimic nasal polyposis, histologic examination of any material removed from the nose should always be done. Surgical removal of melanoma is reportedly the mainstream of therapy [1], [2], [3]. According to our results, a radical (R0) tumour removal is desirable, but mutilating exstirpation does not improve prognosis. We suppose that mutilating surgery is usually not indicated nor is it associated with an improvement of outcome [3], because prognosis is limited by cerebral or pulmonary metastases in most cases. Adjuvant radiotherapy is discussed controversially [2], [3] but it is sometimes reported to have a positive impact. For the limited number of our patients, we can neither confirm nor deny positive effects regarding adjuvant radio-, immuno-, or chemotherapy, when performed immediately after surgery, or in case of tumour recurrence. We conclude from our data and the analysis of literature that therapeutic results in malignant mucosal melanoma of the skull base are primarily based on sufficient surgery. An evidence based multimodal therapy concept does not exist up to now [1], [2], [3]. As the number of patients presenting in one single centre is limited, it is desirable to perform a prospective interdisciplinary investigation on multimodal therapy.


References

1.
Brandwein MS, Rothstein A, Lawson W, et al. Sinunasal melanoma. Arch Otolar Head Neck Surg. 1997;123:290-6.
2.
Rinaldo A, Shaha AR, Patel SG, Ferlito A. Primary mucosal melanoma of the nasal cavity and paranasal sinuses. Acta Otolar. 2001;121:979-82.
3.
Thompson LDR, Wieneke JA, Miettinen M. Sinonasal tract and nasopharyngeal melanoma. Am J Surg Pathol. 2003;27:594-611.