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doi   10.1700/422.5020




Supraclavicular metastases from a sex cord stromal tumor of the ovary

Onur Ismi, Yusuf Vayisoglu, Tuba Karabacak, Murat Ünal
1Department of Otorhinolaryngology, 2Department of Pathology, Mersin University School of Medicine, Mersin, Turkey

Key words: sex cord stromal tumor, ovarian malignancy, neck metastases, supraclavicular lymphadenopathy.

abstract

Metastases to the supraclavicular fossa usually originate from head and neck or infraclavicular tumors. Ovarian primaries of supraclavicular metastases are very rare. Sex cord stromal tumors of the ovary account for 5-8% of all ovarian malignancies and there have been only a few case reports on distant metastases from these tumors. A 46-year-old woman presented to us with a left supraclavicular mass. She had had a sex cord stromal tumor in the right ovary four years before. Comprehensive clinical investigation and fine-needle aspiration cytology were performed. The lesion had the characteristics of a sex cord stromal tumor. To our knowledge, this is the first report of such a case in the English literature. We discuss its pathological and clinical features in the light of the current knowledge.

Introduction
Metastases to the supraclavicular fossa can originate from head and neck and infraclavicular tumors. The principal metastatic sites from the head and neck region are the hypopharynx, tonsil and nasopharynx1. Infraclavicular primary sites are mostly lung, breast and cervix2. Left supraclavicular lymph node enlargement can also occur as a gastric carcinoma metastasis. Ovarian malignancies are rare primary sites for supraclavicular lymphadenopathy.
Sex cord stromal tumors (SCSTs) are generally considered to have a low malignant potential and a favorable prognosis. They are non-epithelial ovarian tumors originating from the sex cords or the stroma and mesenchyme of the ovary. These tumors have been classified as granulosa stromal cell tumors, Sertoli-Leydig cell tumors, gynandroblastoma, sex cord tumor with annular tubules, and unclassified3,4. SCSTs account for 5-8% of all ovarian malignancies. Granulosa cell tumors (GCTs) are low-grade malignancies and account for approximately 70% of SCSTs. SCST may spread by direct local extension or intraperitoneal seeding. Recurrences occur mostly in the pelvis and abdominal cavity. The tumors may also spread hematogenously, with patients developing metastases in the lungs, liver, and brain even years after the initial diagnosis5,6. To our knowledge, there have been no published reports on supraclavicular lymph node metastases from SCST. We present the case of an adult woman who had a GCT in the right ovary with neck metastases, and discuss its pathological and metastatic features in the light of the current knowledge.
Case report
A 46-year-old woman was admitted to our otorhinolaryngology department with a mass in the left neck that had been there for 1 month. She had a history of total abdominal hysterectomy and bilateral salpingo-oophorectomy because of a right ovarian SCST (GCT) 4 years previously. In the ear-nose-throat examination, a 3 × 3.5 cm solid, fixed mass was found in the left supraclavicular region. Endoscopic examination of the upper airway was normal. Computerized tomography (CT) of the neck revealed a 45 × 27 mm lymphadenopathy in the left supraclavicular region (Figure 1). The lymphadenopathy extended into the infraclavicular region. There were multiple metastatic nodules in both lungs. Abdominal CT revealed lymphadenopathies on both sides of the para-aortic region. Fine-needle aspiration cytology from the supraclavicular mass was performed. Homogeneous eosinophilic globular material and oval cells with light chromatin in the nuclei were seen in the cytological specimen (Figure 2). The cells were arranged in a follicular pattern and stained positive with inhibin and CD99 (Figure 3). The pathological diagnosis was in agreement with an SCST. Histologically the lesion was reported to be a GCT and it had the same histological features as the ovarian mass. The patient was referred to the gynecology and oncology department for further therapy. A cisplatinum-based chemotherapy regimen was administered but she died within 2 weeks due to the toxic side effects of this therapy.









Discussion
The differential diagnosis of supraclavicular lymphadenopathy is broad: it includes benign lymphadenopathy, congenital cysts and tumors, specific infections, nonspecific inflammation, primary and metastatic malignancies2. Supraclavicular lymphadenopathy is often malignant (58-83%). It occurs mainly on the left side7,8. Most of the metastases originate from sites other than the head and neck including lung, breast and cervix2,7. Intra-abdominal metastases are also located on the left side and left supraclavicular lymph nodes may be involved in gastric carcinoma metastasis (Virchow’s node)8.
These lesions tend to present as an asymptomatic mass that progresses slowly and is firm on palpation. The associated symptoms are often related to the primary site of the neoplasm and include odynophagia, dysphonia, otalgia and weight loss. Cervical lymph node metastases are a common feature in head and neck malignancies and infraclavicular tumors. The majority of patients with metastases in the lower neck (supraclavicular fossa and posterior triangle) have squamous cell carcinoma. The next most common histological diagnosis is lymphoma, followed by adenocarcinoma and undifferentiated carcinoma 9. Other occult malignant neoplasms such as melanomas, sarcomas and germ cell tumors rarely metastasize to the neck. When the enlarged nodes are in the supraclavicular region, the digestive tract, the tracheobronchial tree, the breast, the genitourinary tract and the thyroid gland should be taken into consideration as possible primary sites10,11. 
SCSTs are histologically considered to be malignant. GCT is the most common among these tumors. GCTs are divided into 2 subgroups based on clinical presentation and histological characteristics: juvenile and adult GCTs, the former accounting for only 5% of cases. Immunohistochemical staining for inhibin may be of help in the pathological diagnosis of GCTs, as this characteristic is very rarely present in other ovarian lesions12.
Different biological behaviors of sex cord tumor have been described in the literature. There are reported cases of lung, liver, brain, bone-skeleton, diaphragm, abdominal wall, and adrenal gland metastases from SCSTs13-17. Lymphadenopathy in the neck is an unusual presentation of malignant neoplasms of the ovary. Only a few cases have been reported of neck metastases associated with ovarian malignancies. In a series of 100 autopsies of women who died of ovarian carcinoma, supraclavicular lymphadenopathy was found in only 4%18. Table 1 gives a summary of case reports for ovarian tumors with cervical metastases. Although SCSTs are generally considered to have low malignant potential and a favorable prognosis, they may generate distant metastases. Since distant metastases can occur as late as 30 years after the initial diagnosis, patients must be followed up for many years even when the primary tumor is occult4,5. In the present case, supraclavicular metastasis occurred 4 years after the primary tumor.

Table 1 - A brief review of the current case reports for ovarian tumors with cervical metastases

Author

Year

No. of cases

Pathology

 

 

 

 

Malpica et al.11

2001

5

Serous tumor

 

 

 

(low malignancy)

Gupta et al.8

2003

9

Adenocarcinoma

Euscher et al.19

2004

8

Serous carcinoma

Mayadevi et al.20

2005

1

Adenocarcinoma

Verbruggen et al.10

2006

3

Serous borderline tumor

Ismi et al.*

2007

1

Granulosa cell tumor

 

 

 

 

*Current report

 

 

 


Fine-needle aspiration biopsy for cervical lymph nodes has 92.7% sensitivity and 98.5% specificity for the primary site. Recent studies using fine-needle aspiration reported the ovary as the primary site in 0.8-9% of cases of supraclavicular lymph nodes7,8.
Since very few cases of distant metastases from SCSTs have been reported in the literature, there is no well-defined treatment protocol. The low incidence of SCSTs, their multiple histological patterns, and their variable biological behavior limit our knowledge regarding the optimal management of these tumors. Surgery remains the most effective treatment for ovarian stromal tumors. SCSTs are characterized by a long natural history and a tendency to recur years after the initial diagnosis. Although the role of postoperative chemotherapy and radiotherapy is not well defined, these treatment modalities have in some cases been associated with prolonged disease-free and overall survival4-6.
Conclusion
Although ovarian cancer rarely metastasizes to the cervical lymph node chain, it should always be considered in the differential diagnosis of lymph node enlargement in the cervical area of adult women.

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