Melanoma of the head and neck: current concepts in staging, diagnosis, and management

Major advances in the understanding of the causes and risk factors for melanoma and for the prevention and management of this tumor have taken place since the beginning of the past century, when the diagnosis of melanoma was synonymous with death. As many as 80% of early melanomas can be cured, and a high rate of locoregional control for even far-advanced melanoma is plausible. The major challenge for the years to come lies in curtailing the steady rise in the incidence of melanoma by increasing patient education and adopting measures to prevent the increasing mortality rates associated with this disease. Cure rates can be improved by early diagnosis by physicians and instant referral to experienced oncologists. Finally, new advances in diagnostic and treatment strategies carry the hope for further improvements in locoregional control and survival rates.     

Paragangliomas of the head and neck

Paragangliomas are uncommon vascular tumours of the head and neck. Eighteen such cases were treated during a six year period. Of the five patients having glomus tympanicum tumours, 3 required a tympanotomy, one a transmastoid excision while one needed a modified Fisch’s type A technique. Eight glomus jugulare tumours were excised by a modified Fisch’s type A approach. One of the four cases with carotid body tumours required carotid replacement by a saphenous vein graft. A glomus vagale tumour was excised transcervically by a median mandibulotomy. Three patients had bilateral tumours. Postoperatively lower cranial nerve palsy persisted in three cases. Two patients having residual intracranial disease received postoperative irradiation. Contrast enhanced CT in axial and coronal planes was found to obviate the need for angiography in all but jugulare tumours. There was no recurrence of symptoms in any case during 6 months to 6 years of followup.     

Schwannomas of the head and neck

Schwannomas are benign encapsulated nerve sheath tumors composed of Schwann cells. Malignant change in head and neck schwannomas is rare, with the incidence varying between 8 and 13.9%. In this review, we discuss the presentation and the management of head and neck schwannomas. The issues and difficulties based on our own experience as well as the experience of published reports from the literature are presented.     

Hamartoma of the head and neck

Hamartoma is a relatively uncommon lesion in head and neck region. Although hamartoma is not a true neoplasm, but it behaves in tumour like manner in many ways. In this paper five cases of hamartoma of head and neck region, one case each from soft tissue cheek, soft tissue neck, maxilla, gum margin and tongue are reported and pertinent literature is reviewed.     

Paragangliomas of the head and neck

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently neighboring vascular structures. Physiologic activity is rare in these neoplasms and they may exhibit patterns of inheritance which predispose their occurrence in families, often with multicentricity. These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties. Other rare sites where this tumor may occur include; the larynx, sinonasal chambers and orbit. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Magnetic resonance represents the most important imaging modality for the evaluation and characterization of suspected head and neck paraganglioma. Definitive management for these lesions should be carefully considered in relation to both tumor and patient-oriented factors, especially in regard to the potential morbidity of treatment. Surgery and radiation therapy represent the main treatment modalities for paraganglioma. The selection of treatment depends on the size, location, and biologic activity of the tumor as well as the overall fitness of the patient. Although radiotherapy may be effective in arresting growth of these tumors, rarely is the neoplasm eliminated without surgical resection. Surgery may be associated with significant morbidity, primarily as a consequence of incurring major cranial nerve injury. Patient selection (relative to age and medical condition) should be carefully considered prior to recommending aggressive surgery for paragangliomas of head and neck, especially in those patients at risk for disabling surgical morbidity.     

Sarcomas of the head and neck

Sarcomas of the head and neck are extremely rare, accounting for less than 1% of all neoplasms of the head and neck. These sarcomas arise in both soft tissues and bone and thus cannot be treated by a single approach. The clinical behavior of these tumors varies considerably. Patients with low-grade lesions are prone to local recurrence, whereas those with high-grade lesions develop both local recurrence and disseminated disease. An additional complicating factor is the multiple sites in which sarcomas may arise in the head and neck. All of these factors make it difficult to ascertain the optimal treatment approach for sarcomas. This article reviews the current literature (as well as the author’s own experience) and provides a general treatment guideline for sarcomas of the head and neck.     

Sarcomas of the head and neck

With the exception of pediatric RMS, soft tissue sarcomas only rarely arise in the head and neck region. Soft tissue sarcomas include a diverse array of histologic types because of the variety of mesenchymal tissues from which they originate. The combination of infrequent occurrence, varied pathologic features, and the many potential sites of presentation makes these tumors a challenge for the head and neck oncologist and underscore the need for review by a pathologist experienced with soft tissue tumors. Classification schemes that group sarcomas according to grade have been helpful in providing prognostic information. Although local control of the primary tumor is critical to successful treatment of both high- and low-grade lesions, the high rate of distant metastases in high-grade tumors supports the role of combined modality therapy. Compared with other types of head and neck neoplasms, such as squamous cell carcinoma, soft tissue sarcomas have low rates of regional metastases. Surgery generally has been recommended as the primary method of treatment for achieving local control, except in those high-grade tumors arising in sites not amenable to resection. Exceptions to this principle include RMSs of the orbit, paranasal sinuses, and masticator space in children; these are usually treated with radiotherapy and combined multiagent chemotherapy, thereby avoiding the functional and cosmetic impact of surgery. Also, extensive angiosarcomas of the scalp should be treated with multimodality therapy combining surgery and wide-field radiation therapy in an attempt to achieve local control. Adjuvant radiotherapy is generally recommended for high-grade sarcomas, large tumors, close or positive surgical margins, and certain histologic variants. Systemic chemotherapy is recommended for those tumors with a significant risk of distant metastases. Increasingly, neoadjuvant chemotherapy is being used to determine responsiveness to chemotherapy, which can help physicians select patients who may benefit from systemic postoperative therapy. Traditional predictors of treatment failure for soft tissue sarcomas include larger tumor size, high-grade histology, and positive surgical margins. The advent of more advanced reconstructive techniques, including free tissue transfer, has made more aggressive surgical resection of these tumors possible. Nevertheless, a considerable number of ancillary support staff are critical to the patient's postoperative rehabilitation and eventual return to a satisfactory level of function and quality of life. In the future, the discovery of the molecular pathogenesis of specific tumor types, such as the cytogenetic findings in synovial sarcoma, will improve physicians' prognostic abilities and selection of patients who are most likely to benefit from emerging adjuvant therapies.     

Paragangliomas of the head and neck

Paragangliomas (PGs) are rare tumors of the head and neck that require a multidisciplinary approach to their management. The recent elucidation of the genetic and molecular etiology for this disease has led to significant advances in our understanding of this disease and has had an important clinical impact. Consideration of genetic testing is strongly suggested for selected patients and their family members. Surgical therapy remains the treatment of choice for most tumors, although a role for stereotactic radiotherapy is emerging. This review highlights some of the advances made in recent years regarding the diagnosis and treatment of PG.     

Lymphomas of the head and neck.

Lymphomas of the head and neck arise in Waldeyer's ring, the salivary glands, nasal cavity, paranasal sinuses, thyroid gland, and orbit. Though anatomically in close proximity, lymphomas arising in these sites have distinct clinical characteristics. Factors that appear to influence the pattern of disease include concurrent conditions, such as Sj?gren's syndrome, and geographic factors, particularly with regard to nasal lymphomas. The treatment and prognosis of patients with head and neck lymphoma depends on the histologic grade of disease and extent of involvement at time of presentation. Most lymphomas are of intermediate-grade histology and early stage at presentation. A thorough understanding of clinical disease patterns and treatment options will allow the optimum management of these patients.     

Mesenchymal chondrosarcoma of the head and neck

Mesenchymal chondrosarcoma of the head and neck is an uncommon tumor with a potential for exhibiting highly aggressive behavior. When these tumors arise in the head and neck region, they appear to have a predilection for the maxillofacial skeleton; less often, they may involve other soft tissue sites in the head and neck. The diagnosis is challenging and may be assisted by molecular pathologic techniques when only limited tissue is available for analysis. Management is primarily surgical. Although adjuvant radiation appears to convey some benefit by reducing tumor bulk when these lesions have extended beyond bony confines, there is no evidence to suggest that this is associated with improved outcome. Chemotherapy does not appear to be effective in the limited experience documented thus far. Patients with complete local control following resection should be followed closely for development of distant metastasis, which signifies a worse clinical outcome. Future effective therapy may be found in the identification of molecular targets responsive to adjuvant chemotherapy or biologic modifiers.     

Malignant melanomas of the head and neck

Presented herewith are eight cases of malignant melanomas of the nose, paranasal sinuses, pharynx and ear.     

Hydatid disease of the head and neck

Two cases of hydatid cyst are reported, one in the right parotid region involving the deep lobe and the other in the superficial subcutaneous tissue of the sub-mental region. Cystic swellings, where no definite preoperative diagnosis can be arrived at, call for a high suspicion rate for hydatid disease and, even in the nonendemic areas, Casoni’s test and complement fixation tests should be carried out.     

Desmoplastic melanoma of the head and neck

The pathologic and clinical findings in cases of desmoplastic melanoma of the head and neck seen at the Mayo Clinic in Rochester, Minnesota, during the past 20 years were analyzed. The nine cases acceptable for study included six usual and three neurotropic variants as defined by light microscopic criteria. Immunopathologic studies of all cases using S-100 protein and desmin, and electron microscopy in four cases, were unsuccessful in distinguishing between the two subtypes. Clinical behavior was aggressive regardless of histologic subtype. Extensive surgical treatment when the diagnosis is made is advocated. The combination of the rarity and the subtle histologic features of desmoplastic melanoma continues to make the correct diagnosis of this entity a challenge for the clinician and pathologist.     

Cutaneous angiosarcoma of the head and neck

Between 1920 and 1970, 13 patients (7 men and 6 women) with cutaneous angiosarcomas of the head or neck were treated at the Mayo Clinic; the mean age of the patients was 66 years. Three clinical patterns were noted: a superficial spreading type, a nodular type, and an ulcerating type. There were no predisposing benign lesions; however, in one patient, angiosarcoma developed in an area of previously irradiated skin. Three modes of therapy were used: surgery alone, radiation alone, and surgery with postoperative irradiation. Of the 13 patients, 2 survived more than 5 years and 2 were alive within 1 year of therapy. Because cervical lymph node spread is common, lymph node clearance is recommended for patients with lateralized lesion or with palpable lymphadenopathy at presentation.     

Cutaneous melanoma of the head and neck

Seventy patients surgically treated in The Netherlands Cancer Institute between 1969 and 1984 for cutaneous melanoma of the head and neck were reviewed with regard to patient data, tumor site, stage, histological criteria, treatment, disease-control and survival. The objectives of the study were to analyse the results of curative treatment of cutaneous melanoma of the head and neck, the value of prognostic factors and the treatment policy for the N0 and N+ neck. Tumor thickness (Breslow Index) was by far the most important prognostic factor in cutaneous melanoma of the head and neck. Other known important factors like level of invasion, tumor subsite, stage, tumor type and ulceration provided additional information. Elective node dissection is advised in lesions thicker than 1.5 mm since N0-N+ transformation is seen in 37% of these patients. Partial neck dissection which includes removal of the nodes adjacent to the primary provides proper regional control except for primary lesions in the neck that require at least a modified neck dissection.