Oncocytoma

We report the case of a 93 year old female patient with a malignant oncocytoma of the parotid gland. Only 32 of these cases have been reported in the world literature so far. Our patient suffered from a 8 x 7 x 6 cm large tumor of the left parotid gland and had a complete facial nerve paralysis. Due to the patient's age and her poor general condition, treatment was intended for palliation only and consisted of total parotidectomy. The patient died nine months later without evidence of recurrent disease. Analysis of the world literature reveals that treatment should consist of surgical tumor removal and neck dissection. The benefit of radiotherapy remains unclear. The 5-year survival is poor due to metastatic disease.

Malignant oncocytomas are very rare tumors of the parotid gland. Only 32 cases have been reported in the world literature so far. We report on a further case in a 93 year-old woman who was in a poor general condition and therefore received treatment primarily for palliation. In order to diagnose malignant oncocytoma, both histological and clinical criteria have to be met. According to the literature, treatment should consist of surgical tumor removal and neck dissection in cases where lymph nodes are presumably affected. The benefit of postoperative radiotherapy is controversial. The five-year survival is poor due to metastatic disease.

Case report

A ninety-three year old female was referred to the Dept. of Otolaryngology, Head and Neck Surgery of the University of Kiel, Germany, from her nursing home for a swelling of the left cheek on 8/27/96. It had been noticed for five years but had grown more rapidly over the last three months. The patient was in a poor general condition and not oriented to time and place. She had no complaints and denied any pain.

Physical examination showed a 8 x 7 x 6 cm large tumor of the left parotid region which was not movable. The overlying skin was intact (Figure 1). There was a complete peripheral facial nerve paralysis. Ultrasound studies revealed a large cystic tumor. There were no enlarged lymph nodes. Magnetic resonance imaging studies showed a 6.6 x 5.5 x 6 cm large tumor of the left parotid gland with involvement of the deep portions (Figures 2a, b). Several fluid-filled areas were present. Again, no enlarged lymph nodes were found.

Total parotidectomy was performed on 9/2/1996. Intraoperatively, a large, hard and non-movable tumor was found with a diffusely infiltrating growth (Figure 3). The facial nerve was atrophic and completely surrounded by the tumor. It was also resected. Reconstruction was not attempted given the age of the patient and her poor health. For palliation, tarsorraphy was performed. The postoperative course was unremarkable. Considering the patient's age, radiotherapy was not applied. The patient died nine months later of cardiorespiratory failure. At this point there was neither evidence of local disease nor pulmonal metastatic disease on a chest X-ray.

Histopathological studies

Macroscopically, a 8 x 7 x 5 cm large parotid gland was found which was encapsulated on one side. Microscopy revealed almost complete infiltration of the gland by oncocytotically transformed cells with a finely granulated cytoplasm, low to intermediate nuclear polymorphy and few mitoses. The oncocytes showed a trabecular structure. The cells showed ductal differentiation and infiltrative growth (Figure 4). A large cyst filled with colloid was lined by oncocytotic epithelium. The capsule of the gland was free of tumor. Vascular invasion and perineural infiltration were present (Figure 5). The final histropathological diagnisis was highly differentiated oncocytic carcinoma of the parotid gland. The TNM-stage was pT4N0M0.

Discussion

Oncocytic carcinomas are extremely rare tumors of the parotid gland. Only 34 cases have been reported in the literature so far (Table 1). The first case was described in 1953 by Bauer and Bauer (1953). Oncocytic carcinomas and their benign variant, oncocytoma, can occur in a variety of organs such as the kidneys, the adrenal glands, the thyroid and the pituitary glands (Chang and Harawi 1992). These tumours are also found in the other large and small salivary glands although even less frequently (Eneroth 1965, Cohen and Batsakis 1968, Ellis et al 1991, Brandwein and Huvos 1991). Three cases of malignant oncocytoma have been reported in the submandibular gland (Sikorawa 1957, Goode and Corio 1988, Ziegler et al 1992). Patients with oncocytic carcinoma are most often in their sixth to eighth decade of life (Hartwick et al 1990, Ellis et al 1991). The patient reported here was, thus far, beyond this range and the oldest with this tumor described so far in the world literature.

Clinical presentation

The clinical presentation of malignant oncocytomas does not differ from that of other tumors of the salivary glands. Sometimes a long past history followed by a sudden growth indicates a preexisting benign oncocytoma (Ellis et al 1991). This may apply to the patient described in the present report. Clinical courses of up to 19 years have been reported (Ellis et al 1991).

Histopathological diagnosis

The differentiation between benign oncocytoma and oncocytic carcinoma has to largely rely on clinical grounds since malignant oncocytoma does not have sufficient histological differences (Batsakis 1974, Goode and Corio 1988).

In general, oncocytes of both benign and malignant tumors present as large cells with a brightly staining, eosinophilic, granular cytoplasm and sharply outlined cell borders. The nuclei can be described as small, rounded and often darkly staining (Mair and Johannessen 1972). The term oncocyte was introduced in 1931 by Hamperl and is derived from the Greek work ovKog for swollen or bulky (Mahmoud 1979). It has been postulated that oncocytes originate from the multipotential cells of the intercalated ducts due to the transition of the small basophilic cuboidal lining cells into swollen acidophilic granular cells (Meza-Chaves 1949, Bauer and Bauer 1953, Evans and Cruickshank 1970, Chu and Strawitz 1978). The tumor cells can appear as solid or glandular formations (Seifert 1996). Up to 60% of the cytoplasm of oncocytes is taken up by mitochondria (Carlsoo et al 1979) which are increased in size and have been shown to be defective in their oxidative phosphorylation which is loosely decoupled (Hubner et al 1967, Askew et al 1971). The ratio of high energy phosphate produced to oxygen consumed is therefore lowered (Schiefer et al 1986). The mitochondroa of oncocytes thus produce only small quantities of ATP (Wackym et al 1986).

In some cases, histology may show indications of malignancy such as pleomorphia of the large, mostly eccentric nuclei with prominent nucleoli and an increased mitotic activity (Haberman et al 1990, Brandwein and Huvos 1991, Ellis et al 1991), despite what has been said above. However, in order to establish a diagnosis of malignancy, one of the following criteria should be met: 1. distant metastasis; 2. local lymph node metastasis; 3. perineural, intravascular or lymphatic spread (as in the case described here); 4. frequent mitosis and cellular pleomorphism with extensive invasion of adjacent structures (Gray et al 1976, Goode and Corio 1988, Mikhail et al 1988, Hartwick et al 1990).

Therapy

Therapeutic recommendations are limited and based on the rather small number of cases described in the literature. The primary treatment of malignant oncocytoma of the parotid gland should consist of wide excision with total parotidectomy (Chu and Strawitz 1978, Goode and Corio 1988, Scher et al 1991). The facial nerve should be resected and grafting should be performed in cases where the facial nerve is paretic preoperatively, due to the tumor, or when it is adherent to the tumor intraoperatively (Harker 1977). Neck dissection has been recommended for all patients (Johns et al 1977, Scher et al 1991) or at least in cases where enlarged local regional lymph nodes can be detected (Harker 1977, Chu and Strawitz 1978). An aggressive surgical approach has been associated with a favourable survival (Goode and Corio 1988). Some authors recommend postoperative radiotherapy (Chu and Strawitz 1978, Scher et al 1991). The patient described in this report was a 93-year old multimorbid nursing home resident in a decreased mental state. Treatment, therefore, was primarily aimed at palliation and no consideration was given to performing neck dissection or postoperative radiotherapy.

Prognosis

Any prognostic statement can only be a gross estimation due to the low incidence of these tumors (Johns et al 1973). Literature data indicates that oncocytic carcinomas smaller than 2 cm offer a better prognosis (Goode and Corio 1988). Although the short term prognosis seems good, the 5 year survival is poor (Goode and Corio 1988, Seifert 1996), often due to metastases which, on average, appear after an interval of 3 years (Ellis et al 1991) Recurrences are found in 2550% (Brandwein and Huvos 1991, Sugimoto et al 1993).

Metastasis, mostly to local lymph nodes, occurs in 50-60% (Brandwein and Huvos 1991, Ellis et al 1991). These cases take a lethal course in 20-40% within one to nine years (Brandwein and Huvos 1991, Sugimoto et al 1993).

Conclusion

Oncocytic carcinomas are very rare malignancies in the parotid gland. The case of a 93 year old female patient is described to add to the 34 cases reported in the world literature. Our patient only received palliative treatment by complete radical surgical excision with resection of the facial nerve. No postoperative radiotherapy was applied, given the poor general condition of the patient. Generally neck dissection should be indicated in larger primary tumors and in cases with lymph nodes detected by ultrasound. Although there is no extensive experience, postoperative radiotherapy is a further therapeutic option which should be pursued, particularly when considering the poor long term prognosis of this disease which, in most cases, is due to metastasis.

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Dr. med. Christoph G. Mahnke

Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Germany Dr. med Ute Janig

Institute of Paedopathology, University of Kiel, Germany Priv.-Doz. Dr. med. Jochen A. Werner Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Germany

Correspondence

J.A, Werner, MD

Dept of Otorhinolarngology, Head and Neck Surgery

University of Kiel

Arnold-Heller-Str. 14

D-24105 Kiel

Germany

Fax: +49-431-597-2272 Tel: +49-431-597-2241 e-mail: werner@hno.uni-kiel.de

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