Cystosarcoma phyllodes metastatic to the mandible: report of a rare case and literature review. (Original Article).

Abstract

Cystosarcoma phyllodes is a rare breast tumor with variable malignant potential. Metastasis has been reported in a small percentage of cases. We describe the case of a 52-year-old woman who developed a large facial tumor 1 year after she had undergone a mastectomy for a rapidly enlarging breast neoplasm. The facial lesion was found to be a malignant cystosarcoma phyllodes metastatic to the mandible, and the patient died shortly after diagnosis. To our knowledge, this patient represents only the third reported case of a phyllodes tumor metastatic to the mandible.

Introduction

Cystosarcoma phyllodes is a rare breast neoplasm that was first described by Muller in 1838. (1) Muller called the tumor cystosarcoma because of its fleshy gross appearance and cystic tendency and phyllodes (leaf-like) because when sectioned, the appearance of its fibrous components resembles that of the veins of a leaf. Muller considered these tumors to be benign, and it was not until nearly 100 years had passed that Lee and Pack described the first case of metastatic cystosarcoma phyllodes in 1931. (1)

Cystosarcoma phyllodes accounts for only 0.5% of all breast tumors. (2) The metastatic potential of this tumor is low, as only approximately 10% of patients develop distant disease. (3) In this article, we describe what we believe is only the third reported case of a malignant cystosarcoma phyllodes that metastasized to the mandible.

Case report

A 52-year-old woman came to the emergency room with a 3-week history of a rapidly expanding mass over the left mandibular angle. She reported no pain or trismus, but she had noticed a decreased sensation in her left lower lip in addition to low-grade fevers and night sweats. One year earlier, she had undergone a mastectomy for a rapidly enlarging breast neoplasm. The pathology report following that surgery described the breast lesion as a malignant cystosarcoma phyllodes.

On physical examination, the patient was pleasant and in no particular distress. Her facial mass was firm, nontender, fixed, and measured 8 x 8 cm. The lesion extended from the angle of the mandible to the submandibular region. Examination of the oral cavity revealed that the tumor had medially displaced the buccal mucosa and mandibular molars. The VIIth cranial nerve was intact, but the mental distribution of the trigeminal nerve was hypoesthetic on the left. Computed tomography (CT) revealed that the mass had destroyed part of the left mandibular body and angle and extended into the submandibular region (figure). Findings on fine-needle aspiration biopsy were nondiagnostic.

The patient was taken to the operating room and given local anesthesia. An open biopsy was performed on the intraoral portion of the tumor, which was easily accessed. The tumor was fleshy and bled quite briskly on incision. Findings on frozen-section analysis were consistent with a metastatic sarcoma. The final pathology report identified the tumor as a metastatic malignant cystosarcoma phyllodes. Follow-up CT of the head, chest, abdomen, and pelvis detected multiple spinal and pelvic metastases as well as a suspected metastatic lesion in the left adrenal gland.

The patient and her family were informed of the diagnosis and the treatment options. Given the dismal prognosis, the patient elected to undergo palliative therapy with radiation to the mandibular metastasis and to the largest of the spinal metastases. Unfortunately, the tumor continued to spread despite treatment, and the patient died of her disease shortly thereafter.

Discussion

The term malignant cystosarcoma phyllodes has been applied to lesions that demonstrate a malignant cellular nature on microscopic examination. Specifically, such tumors feature nuclear atypia, a high mitotic index, a high degree of cellularity, and microscopic invasion of surrounding tissues. Based on these criteria, approximately 16 to 30% of phyllodes tumors are considered to be malignant. (4)

Histologically, malignant lesions have been reported to metastasize in as many as 50% of cases. (5) In a very thorough review, Kessinger et al detailed distant spread in 67 patients with metastatic cystosarcoma phyllodes. (2) The most common sites of metastasis were the lungs (66% of patients), bone (28%), and heart (9%); a gingival metastasis was also noted. Metastasis to the jaw and oral cavity is exceedingly rare. In 1988, Abemayor et al described a case that was nearly identical to ours in their thorough review of the literature. (6) The same year, Tenzer et a1 (7) reported a maxillary metastasis and Cooney et al (8) reported an oral metastasis. In 1991, Yoshimura et al reported a metastasis to the mandibular alveolus. (9) While mandibular metastases of cystosarcoma phyllodes tumors are exceptionally rare, metastases of primary breast adenocarcinomas to the mandible are quite common. (10)

As is the case with other metastatic sarcomas, the prognosis for patients with metastatic cystosarcoma phyllodes is bleak, and treatment options are limited. Although some cases of long-term survival have been reported following surgical resection of isolated metastases, such an outcome appears to be the exception rather than the rule. (11,12) Because many of these patients have unresectable or widespread disease, chemotherapy and radiation have been used as both curative and palliative therapy. Long-term remission has been achieved on occasion, but the overall results have been disappointing. (13) Because the number of reported cases is very small, comparisons of treatment modalities are difficult to make.

References

(1.) Lee BJ, Pack GT. Giant intracanalicular myxoma of the breast. The so-called cystosarcoma phyllodes mammae of Johannes Muller. Ann Surg 193 1;93:250-68.

(2.) Kessinger A, Foley JF, Lemon HM, Miller DM. Metastatic cystosarcoma phyllodes: A case report and review of the literature. J Surg Oncol 1972;4:131-47.

(3.) Pietruszka M, Barnes L. Cystosarcoma phyllodes: A clinicopathologic analysis of 42 cases. Cancer 1978;41:1974-83.

(4.) Rosenfeld JC. DeLaurentis DA, Lerner H. Cystosarcoma phyllodes. Diagnosis and management. Cancer Clin Trials 1981;4:187-93.

(5.) Treves N, Sunderland DA. Cystosarcoma phyllodes of the breast: A malignant and benign tumor. Cancer 1951;4:1286-332.

(6.) Abemayor E, Nast CC, Kessler DJ. Cystosarcoma phyllodes metastatic to the mandible. J Surg Oncol 1988;39:235-40.

(7.) Tenzer JA, Rypins RD, Jakowatz JG. Malignant cystosarcoma phyllodes metastatic to the maxilla. J Oral Maxillofac Surg 1988;46:80-2.

(8.) Cooney BM, Ruth GJ, Behrman DA, et al. Malignant cystosarcoma phyllodes of the breast metastatic to the oral cavity: Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1988;66:599-604.

(9.) Yoshimura Y, Inoue Y, Mihara Y, Miura H. Metastatic malignant cystosarcoma phyllodes. Report of a case with an oral tumour and review of the literature. J Craniomaxillofac Surg 1991; 19:227-31.

(10.) Epker BN, Merrill RG, Henny FA. Breast adenocarcinoma metastatic to the mandible. Report of seven cases. Oral Surg Oral Med Oral Pathol 1969;28:471-9.

(11.) Fernandez BB, Hernandez FJ, Spindler W. Metastatic cystosarcoma phyllodes: A light and electron microscopic study. Cancer 1976;37: 1737-46.

(12.) Hart WR, Bauer RC, Oberman HA. Cystosarcoma phyllodes. A clinicopathologic study of twenty-six hypercellular periductal stromal tumors of the breast. Am I Clin Pathol 1978;70:211-16.

(13.) Hawkins RE, Schofield JB. Wiltahaw E, et al. Ifosfamide is an active drug for chemotherapy of metastatic cystosarcoma phyllodes. Cancer 1992;69:2271-5.

From the Department of Otolaryngology--Head and Neck Surgery, University of Tennessee, Memphis (Dr. Staton and Dr. Costello), and the Department of Radiology, Methodist University Hospital, Memphis (Dr. Donovan and Dr. Laster).

Reprint requests: Jonathan Staton, MD, Department of Otolaryngology--Head and Neck Surgery, University of Tennessee, 956 Court Ave., Room B226, Memphis, TN 38163. Phone: [phone]; fax: [phone]; e-mail: [e-mail]