Journal of Neuro-Ophthalmology, March 1991, Volume 11, Issue 1

Intracranial extension of meibomian gland carcinoma.

Meibomian gland carcinomas are rare, slow-growing masses that clinically mimic several benign and malignant processes. We report a patient with sebaceous carcinoma of the meibomian gland with intracranial extension and dural invasion, and discuss the clinical and pathological features of this uncommon entity.

Journal of C1illiCQI Neuro- ophthalmology 11( 1): 39- 42, 1991. Intracranial Extension of Meibomian Gland Carcinoma Ahmet <;;= olak, M, D., Cern Akkurt, M, D., Osman Ekin Ozcan, and Behsan Onol ,[; j 1991 Raven Press, Ltd., New York Meibomian gland carcinomas are rare, slow- growing masses that clinically mimic several benign and malig­nant processes. We report a patient with sebaceous car­cinoma of the meibomian gland with intracranial exten­sion and dural invasion, and discuss the clinical and pathological features of this uncommon entity. Key Words: Dural invasion- Intracranial extension­Meibomian gland carcinoma- Sebaceous carcinoma. From the Departments of Neurosurgery ( A. C., C. A., O. E. O.) and Pathology ( B. a.), Hacettepe University School of Medi­cine, Ankara, Turkey. Address correspondence and reprint requests to Dr. Ahmet <;: olak, Tip Fakiiltesi Caddesi, 36/ 5, Abidinpa § a, Ankara, Tur-key. r-----------" 39 Meibomian gland carcinomas of the eyelid are rare neoplasms, accounting for less than 1% of all eyelid tumors ( 1- 4). Most of the cases mimic cha­lazia and undergo repeated curettage before a de­finitive diagnosis is confirmed ( 2- 4). Although lo­cal recurrence and regional lymph node me­tastases are common, distant metastases are extremely rare ( 2,3,5). Only a few cases have been reported previously. One had seeding in the intra­cranial soft tissue, and metastasized to the cerebel­lum ( 1,4- 6). In this report, a meibomian gland carcinoma in­vading the frontal bone and adjacent dura mater is presented. We are not aware of previously re­ported sebaceous carcinoma of the meibomian gland involving the frontal bone on the forehead and dura mater. CASE REPORT A 57- year- old woman was admitted to the Neu­rosurgery Department of Hacettepe University Medical School Hospital with the complaint of a painless swelling on the frontal region. From her medical history, we learned that 3 years prior to admission she had noted an upper eyelid reddish swelling that was excised by her local ophthalmologist 2 years previously. No his­topathological examination was done. Fifteen months later the lesion recurred and histopatho­logical examination of a biopsy revealed a seba­ceous carcinoma of the meibomian gland. The mass on the upper eyelid was excised by the Plas­tic Surgery Department, and in a second session, an upper lid was constructed from the lower one using an advancement flap. Physical Examination A mass was present, starting from the root of the nose and reaching the medial edge of the left eye- A. (: OLAK ET AL. blt,\\. On the right, it invaded the orbital rim and frt) ntal b: llit' tln the forehead in the midline and e\. kndl'd tll the middle of the right eyebrow. The m, 1~~ \", 1<; h, Hd, fixed, and 5 x 5 cm in size. The remaindL'l' llf the ncular examination was unre­markable. There were no palpable regional lymph nodes t) r di~ lant metastatic lesions. On neurologi­cal e\, aminatinn, right eye movements were re­stricted for upward and lateral gaze. Other neuro­logical findings including visual acuity and light reflex were normal. Routine laboratory studies such as hemoglobin level, urinary analysis, and liver function tests were within normal limits. Plain roentgenograph of the skull showed a lytic lesion at the region of the mass, Computerized to­mography revealed massive tumor involvement of the frontal sinus and anterior ethmoid sinuses with an intracranial component entering the ante­rior cranial fossa ( Fig. 1) Operation Bifrontal skin incision and following frontal craniectomy were performed. The tumoral mass that was subtotally removed was gray- violet in color and bled easily. It infiltrated the frontal bone on the forehead and dura, and extended toward the subdural space. It also filled the frontal and anterior ethmoid sinuses. Microscopic Examination The surgical specimen contained a mass that ex­tensively invaded the frontal bone and dura mater. Irregular lobular formations with great variation in the size of the lobules separated by fibrous septa FIG. 1. Axial computed tomogram showing intracra­nial extension of the tumor involving the frontal bone. , ,' 1,,, ,'\:"'" o-" I, IIII", lmol, Vol. 11, No. L 1991 were present. Although many . Wt ldiffer-entiated, distinct sebaceous CE' b a foamy cytoplasm were seen in the eel.,'': ot mo~ t l~ bules. Undifferentiated cells had frequent mItoSIS and showed considerable variation in the shape and size of their nuclei and nucleoli. Some of the large lobules showed areas of atypical keratinizing cells, bleeding, and necrosis. These poorly differentiated basoloid cells with sebaceous features arranged centrally in nests characterized the lesion. Slides from the initial biopsy demonstrated identical his­topathological features ( Fig. 2A and B). Postoperative Course The patient's condition was good and she was referred to the Oncology and Plastic and Recon­structive Surgery Departments. Chemotherapy consisting of cis- pIatin, bleomycin, methotrexate, and 5- fluorouracil, and irradiation of 6,000 rad were given. There was no evidence of recurrence or metastasis 6 months after radiation therapy. The patient was still alive 15 months after the last op­eration. DISCUSSION Sebaceous carcinomas of the meibomian gland are rare, accounting for only 1% of all eyelid tu­mors and 4.7% of malignant tumors ( 1- 4). They are slow growing, clinically mimicking several be­nign processes such as chalazion, blepharocon­junctivitis, keratoconjunctivitis, granuloma, or ma­lignant lesions including basal cell and squamous cell carcinoma ( 1,3,4). Fifteen to 30% of patients with sebaceous carcinoma of the eyelid have local lymph node metastases that usually present in the preauricular and cervical lymph nodes ( 2- 5). In our case, no lymph node metastases were ob­served at the time of intracranial extension of the tumor. Although direct involvement of the orbit often occurs, ~ istant metastases spreading from the pri­mary leSIOn to the lungs, liver, brain, base of the skull, and cerebellum can be found in rare cases ( 1- 6). Our review of the literature failed to find another report of a sebaceous carcinoma of the meibomian gland invading the frontal bone and adjacent dura mater and extending to anterior cra­n. i~ 1 fossa. I~ is well known that during the postex­ClsIOnal penod, some malignant tumors may have more aggressive behavior than before. In this case the poorly differentiated tumor cells with areas of necrosis and bleeding were more apparent i" 1 ma­terial obtained from the dura mater and ' ntal MEIBOMIAN CARCINOMA EXTENSION 41 FIG. 2. A: Initial excisional biopsy con­firmed the diagnosis of sebaceous car­cinoma of the meibomian gland ( H& E x230). B: Photomicrograph of recur­rent tumor. Tumor cells are pleomor­phic with diffuse growth pattern, prom­inent nucleoli, and foamy vesiculated cytoplasm. ( H& E x460j FIG. 3. Microscopic examination show­ing extensive infiltration of the dura mater by the tumor. ( H& E x460) JClin Neuro- ophthalmol. Vol. II, No. 1, 1991 42 A C; OLAK ET AL. bone than in the initial biopsy material ( Figs. 2 and 3). Involvement of the frontal bone and dura mater can be explained by this aggressive behavior. Russel et al. ( 7) noted that the pagetoid spread of neoplastic cells was the most important diagnostic and prognostic data in these cases. There was no typical pagetoid spread in the present case. The only effective therapeutic approach for se­baceous carcinoma of the meibomian gland is rad­ical surgery; the value of radiation and chemother­apy is minimal ( 1- 4,8). When there is intracranial extension, it is difficult to treat and prognosis is poor. We believe that total resection, facilitated by early detection prior to intracranial extension, is mandatory for successful treatment of this clinical entity. We also stress that a surgeon should take this entity into consideration when dealing with a lesion located on the forehead and extending in­tracranially. REFERENCES 1. Boniuk M, Zimmennan LE. Sebaceous carcinomas of the eyelid, eyebrow, caruncle, and orbit. Trans Am Acad Oph­thalmoIOtolaryngoI1968; 72: 619- 22. 2. Doxanes TM, Green RW. Sebaceous gland carcinoma. Re­view of 40 cases. Arch Ophthalmol 1984; 102: 245- 9. 3. Rao NN, Hidayat AA, McLean IW, et aI. Sebaceous carci­noma of the ocular adnexia: a clinicopathological study of 104 cases with five- year follow- up data. Hum Pathol 1982; 13: 113-- 22. 4. Rao NN, McLean IW, Zimmennan LE. Sebaceous carci­noma of the eyelids and caruncle. Correlation of clinical pathologic features with prognosis. In: Jakobiec FA, ed. Ocular and adnexal tumors. New York: Aesculapius Pub. Inc., 1978: 461- 76. 5. Mashburn MA, Chonkich GO, Chase DR, et aI. Meibomian gland adenocarcinoma of the eyelid with preauricular lymph node metastasis. Laryngoscope 1985; 95: 1441- 3. 6. Bryant J. Meibomian gland carcinoma seeding intracranial soft tissue. Hum Pathol 1977; 8: 455- 7. 7. Russel WG, Page DL, Houg JA, et aI. Sebaceous carcinoma of meibomian gland origin. The diagnostic importance of pagetoid spread of neoplastic cells. Am JClin Pathol 1980; 73: 504-- 11. 8. Khalil MK, Lorenzetti HD. Sebaceous gland carcinoma of the lid. Can JOphthalmo/ 1980; 15: 117- 21.