Lumbosacral Chordoma
Metastatic to the Eyelid
TIMOTHY J. MALONE, MD,* ROBERT FOLBERG, MD,t JEFFREY A. NERAD, MD*
Abstract: The authors report a patient treated with radiation therapy for a lumbosacral chordoma. The first and only evidence of metastatic disease was an eyelid cyst. This case is unusual for several reasons. First, eyelid metastases are rare; they are much more uncommon than metastases to the uvea or orbit. Second, metastatic tumors to the eyelid are usually manifestations of widespread disease and seldom occur as a solitary focus of dissemination. Third, the clinical presentation of our patient's lesion as an eyelid cyst is uncommon for a metastasis to the eyelid. Finally, to the best of our knowledge, all previously reported tumors metastatic to the eyelid have been carcinomas; this is the first report of a metastatic soft-tissue tumor to the eyelid. [Key words: chordoma, cyst, eyelid, lumbosacral spine, metastasis.] Ophthalmology 94:966-970,1987
Metastatic eyelid tumors are rare. The incidence of eyelid metastasis among all malignant eyelid neoplasms ranges from 0.6 1 to 1.4%.2 Riley3 found only 13 cases of metastasis to the eyelid at the Mayo Clinic for the years 1922 through 1969. To the best of our knowledge, there are no previous reports of eyelid metastases from malignant soft-tissue tumors. l -4 We report a patient with a primary chordoma of the lumbosacral spine who had a solitary metastasis to the eyelid presenting as a cyst.
CASE REPORT A 65-year-old white man complained of a left lower lid mass that had enlarged over 4 months. His past ocular history was significant only for a successful retinal reattachment in the right eye in 1984. On examination, the visual acuity was 6/9 in both eyes. A 10 X 5 X 5-mm cyst was present in the left lower lid. The cyst transmitted light well and was fixed to the tarsus
From the Oculoplastic, Orbital and Oncology Service," and the Eye Path· ology Laboratory,t University of Iowa Hospitals and Clinics, Iowa City. Reprint requests to Robert Folberg, MD, Eye Pathology Laboratory. University of Iowa Hospitals and Clinics. Iowa City. IA 52242.
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medially. There were no palpable lymph nodes. The anterior segments were normal bilaterally and the retinas were attached. The patient had a 30-year history of progressive lumbar disc degeneration producing neurogenic bladder, rectal incontinence, lower extremity pain, and polyneuropathy. Resection of the discs had been done at L3-4 and L5-S I with partial relief of pain and increased sensation. The patient had required the placement of an indwelling catheter since 1968 and had had recurrent episodes of urosepsis prompting his admission in April 1984. An abdominal computed tomographic (Cf) scan (Fig l) done at that time demonstrated destruction of the body ofL4 by a mass extending locally into the psoas. A needle biopsy of the lesion was done and showed a malignant neoplasm thought to be a carcinoma (Fig 2). A work-up, including intravenous pyelogram, renal CT, and prostatic biopsy did not disclose a primary urogenital tumor as suspected clinically. Radiation therapy consisting of 3500 rad was delivered to L2 through S I with some relief of pain. In April 1986, the patient was readmitted for urinary tract infection. A posterior decompression and drainage of a lumbosacral epidural abscess was done with removal of the posterior element of L2 to S I, resulting in residual severe instability and 50% retrolisthesis of L4 through L5. Results of pathologic examination of the resected tissue showed chordoma (Fig 3). The material removed by open biopsy was consistent in appearance with the needle-biopsy material obtained 1 year earlier. The patient's vertebral lesion was left open to close by granulation and conservative wound care was applied to the
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yielded 0.5 ml of viscous clear material, and on completion of the aspiration blood filled the cyst, which had been only partially collapsed. The residual lid mass was subsequently excised. Results of pathologic examination of the resected tissue showed a cystic cavity lined by cords of cells with bubbly cytoplasm (physaliferous cells; Figs 4, 5). The tumor matrix stained positive for acid mucopolysaccharides. The lid lesion was histologically identical in appearance to the chordoma resected from the lumbosacral spine (Fig 3). A metastatic survey including liver-spleen scan, bone scan, abdominal and cranial CT scans showed no other foci of disease outside the pelvis. Because additional surgical resection and radiotherapy to the primary tumor were considered to be only palliative,5 no further treatment was recommended. The patient was eventually transferred to a chronic care facility.
DISCUSSION
Fig 1. CT scan of the lumbosacral spine demonstrating destruction of the vertebral body and infiltration of a soft tissue mass (open arrows) into adjacent structures. A fistula (f) is present.
An ophthalmic consultation was requested in July 1986 for evaluation of the enlarging left lower eyelid mass. The unstable neurosurgical condition prompted an attempt to drain the lower lid cyst at the bedside; the lesion clinically appeared to be a ductal cyst of eccrine origin. The attempted aspiration
The clinical differential diagnosis of the cystic eyelid lesion in our patient mistakenly focused on cysts of the eyelid sweat glands, although basal cell carcinoma may also present as a cystic lesion of the eyelid. 6 The viscous contents of the cyst in our patient and the failure of the cyst to collapse totally after aspiration were inconsistent with the clinical impression of sudoriferous cyst, prompting an excisional biopsy. The definitive diagnosis was established by pathologic examination, emphasizing the importance of submitting all excised tissue specimens to the laboratory.
Fig 2. Fine-needle biopsy of spinal mass demonstrating a cluster of cells with vacuolated cytoplasm (hematoxylin-eosin; original magnification, X 130).
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Fig 3. Material obtained at open biopsy of the spinal mass. Upper left, notice the cluster of cells with bubbly cytoplasm: physaliferous cells (hematoxylin-eosin; original magnification, X33).
Metastatic chordoma was not considered in the clinical differential diagnosis for several reasons. First, metastatic eyelid tumors are uncommon l ,2 and are generally manifestations of widespread metastatic disease. The manifestation of metastatic disease as a solitary eyelid lesion demonstrated by our patient is very unusual, although similar presentations have been reported in metastatic breast carcinoma. 7 ,8 Second, the clinical presentation of an eyelid metastasis as a cyst is unusual. Riley3 described three characteristic clinical presentations of tumors metastatic to the eyelid: (1) nontender, indurated plaques, characteristic of metastatic breast carcinoma; (2) solitary noninflamed subcutaneous nodules, usually seen with metastatic melanoma to the eyelid; and (3) ulcerating nodules, most commonly seen in metastatic lung and stomach carcinomas. Inflamed eyelid nodules mimicking chalazion have been attributed to metastatic lung carcinoma,4 renal cell carcinoma,9 and breast carcinoma. 10 Finally, all previous reports of metastases to the eyelid have documented carcinomas as the primary tumors. Breast carcinoma is the most common metastatic tumor to the eyelid accounting for between 38 3 and 65%4 of cases. The lung is the second most frequent source of tumors metastatic to the eyelid (15%) followed by the stomach (10%). 4 Other sources of tumors metastatic to the eyelid include the trachea, II the parotid, 12 the kidney (renal cell carcinoma),8 esophagus, and urinary bladder.1 To the best of our knowledge, no malignant softtissue tumors metastatic to the eyelid have been described previously.
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Chordoma is an uncommon neoplasm arising from notochordal remnants. The tumor arises most frequently in the sacrococcygeal region followed by occurrences as primary intracranial tumors of the basiocciput and, less frequently, masses involving other vertebral zones. 13 ,14 Chordoma is generally regarded as a slowgrowing, locally aggressive tumor with low potential for metastasis. There is, however, disagreement on the incidence of metastasis ranging from 312 to 48%15. One literature review estimates the incidence of metastasizing extracranial chordomas at 15%. 13 One source of the discrepancy in the incidence of metastasis from chordoma may be the finding that tumor radiation may predispose to dissemination. 14 In the current case, our patient received 3500 rad 1 year before the appearance of the isolated lid metastasis because the primary tumor was not accurately diagnosed as a chordoma. Additionally, the chordoma in our patient involved the lower vertebral region, and tumors arising in this area are reported to have a higher rate of metastasis than chordomas of the basiocciput. 14,15 The most common sites of metastases by chordoma are the lungs, lymph nodes, the liver, bone, skin, and skeletal muscle. 13 ,14 In one series of metastatic chordomas, three cases of metastases to the skin were reported; the original histopathologic diagnosis on all three of these cases was a mixed tumor of the skin (chondroid syringoma).14 Chondroid syringomas have been reported in the eyelid,16,17 and we considered this entity in the histopathologic differential diagnosis of our patient. Histologically, chondroid syringoma and chor-
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Fig 4. Top. cyst removed from the eyelid, demonstrating metastatic chordoma. Notice the sebaceous glands (arrows), evidence of the location of this metastasis in the skin. The edges of the cyst wall are marked by arrowheads (c = partially collapsed cyst lumen) (hematoxylin-eosin; original magnification, X3). Bottom. a higher magnification of another section through the cyst wall, demonstrating a sebaceous gland (upper left) adjacent to tumor (hematoxylin-eosin; original magnification, X8).
doma may share a stromal matrix that resembles cartilage, but chondroid syringomas lack the physaliferous cells characteristic of chordoma (Fig 5), and chordomas lack the tubular epithelial organization characteristic of chondroid syringoma. 16
Although no cases of eyelid metastasis by chordoma have been reported previously, involvement of the periocular structures by direct extension of intracranial chordoma arising from the clivus has been well documented. 18,19
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Fig S. Higher magnification of the cystic eyelid metastasis. Notice the physaliferous cells (hematoxylineosin; original magnification, X130).
REFERENCES 1. Weiner JM, Henderson PN, Roche J. Metastatic eyelid carcinoma. Am J Ophthalmol1986; 101:252-4. 2. Aurora AL, Blodi FC. Lesions of the eyelids: a clinicopathological study. Surv Ophthalmol 1970; 15:94-104. 3. Riley FC. Metastatic tumors of the eyelids. Am J Ophthalmol 1970; 69:259-64. 4. Amold AC, Bullock JD, Foos RY. Metastatic eyelid carcinoma. Ophthalmology 1985; 92:114-9. 5. Sundaresan N, Galicich JH, Chu FCH, Huvos AG. Spinal chordomas. J Neurosurg 1979; 50:312-9. 6. Petersen RA, Aaberg TM, Smith TR. Solid vs. cystic basal cell epitheliomas of the eyelids: correlation of clinical and pathological diagnoses. Arch Ophthalmol1968; 79:31-2. 7. Lessans SH. Cancer of the breast metastatic to the eyelid alone. Am J Ophthalmol1973; 75:458-60. 8. Amoni SS, Rodrigues MM, Shannon GM. Breast carcinoma metastatic to the eyelid. Can J Ophthalmol 1973; 8:167-70. 9. Kindermann WR, Shields JA, Eiferman RA, et al. Metastatic renal cell carcinoma to the eye and adnexae. Ophthalmology 1981; 88:134750.
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10. Weinstein GW, Goldman IN. Metastatic adenocarcinoma of the breast masquerading as chalazion. Am J Ophthalmol 1963; 56: 690-3. 11. Oltmans HJ. Carcinoma palpebrae metastaticum. Ned Tijdschr Geneeskd 1930; 74:1532-4. I'etude des tumeurs metastatiques des 12. Vrabec F. Contribution paupieres. Ophthalmologica 1951; 122:362-6. 13. Markwalder TM, Markwalder RV, Robert JL, Kmeta A. Metastatic chordoma. Surg Neuro11979; 12:473-8. 14. Chambers PW, Schwinn CPo Chordoma: a clinicopathologic study of metastasis. Am J Clin Patho11979; 72:765-76. 15. Volpe R, Mazabraud A. A clinicopathologic review of 25 cases of chordoma (a pleomorphic and metastasizing neoplasm). Am J Surg Patho11983; 7:161-70.
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16. Hirsch P, Helwig EB. Chondroid syringoma: mixed tumor of the skin, salivary gland type. Arch Dermatol 1961; 84:835-47. 17. Font RL. Eyelids and lacrimal drainage system. In: Spencer WH, Font RL, Green WR, et ai, eds. Ophthalmic Pathology. An Atlas and Textbook. 3rd ed. Philadelphia: WB Saunders Co, 1986; 2215-8. 18. Bagan SM, Hollenhorst RW. Ocular manifestations of intracranial chordomas. Trans Am Ophthalmol Soc 1980; 78:148-55. 19. Ferry AP, Haddad HM, Goldman JL. Orbital invasion by an intracranial chordoma. Am J Ophthalmol1981; 92:7-12.