Medical I ztelligence MEIBOMIAN GLAND 9 CARCINOMA SEEDING I N T R A C R A N I A L SOFT T I S S U E S JAMES BRYANT, *I.D.* Abstract Meibomian gland carcinoma is a rare sebaceous gland malignant tumor of the eyelid. Local recurrence and regional lymph node metastases are common among this group of eyelM [email protected]
Distam liver metastases have been mentiolzed in one case report, but this represents an umtsual flmling. In addition to local recurrence and regional lymph node metastases, this autopsy case report demonstrates meibomian gland carcinoma with extension to the trigeminal ganglia and metastases to the cerebellum. Meibomian gland carcinoma is a rare malignant t u m o r o f the eyelid thougl~t to account for about 1 p e r cent o f all eyelid neoplasnls. ~ Literature reviews by Ginsburg I a n d by Boniuk and Zilnnlerman 2 as well as m o r e recent case reports suggest as many as 185 cases r e p o r t e d in the literature. Although Allaire 3 is generally credited with the first case r e p o r t in 1891, Thiersch 4 may have r e p o r t e d an earlier case. On the basis o f his literature review, Ginsburg ~ found a 32 per cent rect,rrence rate, a 6 p e r cent incidence o f orbital extension, a 17 p e r 9cent rate o f lymph node metastasis, and a 6 per cent mortality rate. One case with distant liver metastases has been r e p o r t e d ? Tiffs publication presents an autopsy case r e p o r t o f meibomian gland carcinoma eroding the orbit, e x t e n d i n g to both trigeminal ganglia, a n d metastasizing to the cerebelluna. T h e r e are no previous reports o f meibomian gland carcinoma involving intracranial soft tissues. CASE R E P O R T
At the age o f 39 the patient went to her physician because o f a small nodule o f the left u p p e r eyelid. Considering the lesion a chalazion, h e r physician d r a i n e d the nodule, but several months later it r e t u r n e d . T h e lesion was excised. Squamous cell carcinoma was the *Assistant. l)el)artmcnt of Pathology, Rush Medic;fl College. Resident in Pathology, l'resbyterian St. Luke's Hospital, Chicago. Prosector, Department of Pathology and l.aboratory Medicine, St. Francis Hospital, Blue Island, Illinois.
histopathologic interpretation. T h e patient r e m a i n e d asymptomatic for four )'ears and f o u r months, but at the age o f 44 she developed a r e c u r r e n t lesion o f the left u p p e r eyelid. A 6000 r a d dose o f radiation caused regression o f the lesion. Exactly one ) e a r later a recurrence was suspected b u t was not clinically obvious for a n o t h e r 10 months. A biopsy diagnosis o f undifferentiated carcinoma suggested orbital exenteration, but tiffs p r o c e d u r e was refilsed by the patient. One )'ear later, in Dec e m b e r 1970, the patient was referred to Rushl'resbyterian-St. Lukes Medical Center in Chicago. Biopsy study showed meibomian gland carcinoma and an orbital exenteration was p e r f o r m e d . T h e patient remained free from disease for 14 months, when a local r e c u r r e n c e a p p e a r e d . This was treated successfully with r a d i u m iml)lants, but approximately six months later she d e v e l o p e d a painfill l u m p in the left u p p e r cheek. T h r e e months later the l u m p was removed a n d histologic study d e m o n strated a buccinator l y m p h node with metastatic carcinoma. After t h r e e months, in J a n u a r y 1973, a left s u p r a o m o h y o i d and superficial cervical lympll node dissection was p e r f o r m e d for recurrent tumor. T w o o f eight lymph nodes demonstrated metastatic disease. Eleven months later she developed a recurrent nodule in her cheek and u n d e r w e n t partial neck dissection a n d partial mandibulectomy. Nine o f 22 lymph nodes showed metastatic tumor. Following surgery she suffered d)'splmgia, weight loss, uncontrolled salivation, and generalized muscle weakness. She died three months postoperatively fi-om aspiration pneunlonm.
PATHOLOGY T h e left orbital exenteration included the u p p e r and lower eyelids, the globe, a n d the associated musculature, fat, and connective tissue. T h e optic nerve, globe, and associated orbital structures were free from tumor. An irregular, firm yellow conjuoctival mass m e a s v r i n g 1.5 by 1.0 by 0.5 cm. was located ill the u p p e r temporal quadrant. Other areas o f the conjunctiva[ tissue showed tlfickening and granularity. Microscopic studies demonstrated a transition from normal meibonaian gland to meibomian gland carcinoma (Fig. I). T h e neoplastic cells f o r m e d r o u n d to oval acinar aggregates s u r r o u n d e d by a basement m e m b r a n e .
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F i g u r e 1. T h e eyelid neoplasm originates from normal lneibomian glands. T h e conjunctival surface is seen in the u p p e r right h a n d corner. Inset: T h e conjtmctiva shows a pagetoid involvement by plcomorphic t u m o r cells with a pale cytoplasm.
Tile cells were r o n n d to spindle slmped a n d cell b o r d e r s were indistinct. Numerous mitotic figures were seen in all acini. T h e conjunctival el)itlleliunl showed pagetoid invasion by well differentiated cells, some o f which lind distinct cell borders (Fig. 1, inset). N u m e r o u s examples o f nerve and blood vessel invasion were seen t h r o u g h o u t tile specimen. At autopsy, necrosis and t u m o r were found in the m o r e medial portions o f the middle cranial fossa. T h e t u m o r was white, friable, and wet and cross section examination showed gross involvement o f the left trigeminal gang l i o n . T h e neoplasnt a p p e a r e d to encroach u p o n the pituitary gland, but actual invasion o f the pituitary p a r c n c h y m a was not grossly obvious. Careftd dissection showed no gross evktenee o f ntetastases in o t h e r body parts. Microscopic examination o f the trigeminal ganglia d e m o n s t r a t e d cords o f neoplastic cells invading the stroma o f the ganglia. T h e left trigeminal ganglion showed much heavier invasion than the right. Partial obliteration o f ganglion cells by the t u n m r was demonstrated in several areas (Fig. 2). T h e cells o f the neoplasm had hyperchromatic pleomorplfic nuclei, indistinct cell borders, a n d a cord arrangement. Extensive areas o f necrosis ringed by t m n o r
cells were seen t h r o u g h o u t the trigeminal ganglia. In addition to small focal collections o f t u m o r cells in its meningeal coverings, the cerebellnm d e m o n s t r a t e d invasion o f the molecular layer (Fig. 2, inset). No m i n o r was seen in the cerebral hemispheres. Discussion Tile case presented ill this r e p o r t demonstrates a highly invasive form o f meibonlian gland carcinoma. In the eleven and one half )'ear course o f tiffs patient's disease, there were eight recurrences o f the t u m o r as well as regional l y m p h node metastases. In the beginning o f its course, the t u m o r grew in a seemingly indolent fashion. However, a f o u r )'ear disease free p e r i o d was followed by a r a p i d rate o f r e c u r r e n c e and lymph node metastases a n d t e r m i n a t e d with erosion o f the orbit a n d spread to the trigeminal ganglia and cerebellum. T h e accelerating malignant potential o f meibomian gland carcinoma has been observed by o t h e r authors a n d is considered to be related to inadequate local excision. 6"z A h h o u g h tile original eyelid lesion in tiffs case was designated squamous cell carcinoma, review o f the original slides demonstrated a neoplasm in the vicinity o f normal meibomian glands with
Figure 2. Invasion of the trigeminal ganglion by round cells with hyl~erchromatic nuclei forming a cord pattern. Erosion of ganglion cells by malignant cells is seen in the lower right hand corner and slightly to the right of the center. Inset: The molecular layer of the cerebellum is invaded by nests of round cells with hyperchromatic nuclei sintilar to those in the trigeminal ganglia. Cells with finely granular nuclei are also present.
histologic f e a t u r e s identical to those o f Figu r e 1. Keratin pearls and i n t e r c e l l u l a r bridges typifying s q u a m o u s cell c a r c i n o m a w e r e not seen a n y w h e r e . As in this case, the diagnosis o f s q u a m o u s cell c a r c i n o m a a n d t m d i f f e r e n t i a t e d c a r c i n o m a has been g i v e n to these t u m o r s by a significant p e r c e n t a g e o f pathologists called u p o n to review eyelid t u m o r s . ~ T h e initial clinical p r e s e n tation in what is a p p a r e n t l y an u p p e r eyelid chalazion f o l l o w e d a short t i m e later by a r e c u r r e n c e is typical o f m e i b o m i a n gland carcinoma. In t h e i r series o f 88 cases B o n i u k and Z i m m e r m a n -~ described a case with brain i n v o l v e m e n t . H o w e v e r , t h e y d i d not classify t h e i r case a c c o r d i n g to the g l a n d o f origin. Zeis and m e i b o m i a n gland c a r c i n o m a s as well as sebaceous g l a n d c a r c i n o m a s o f the c a r u n c l e and e y e b r o w w e r e i n c l u d e d in this series. T h e case p r e s e n t e d h e r e d o c u m e n t s the first rep o r t e d case o f m e i b o m i a n g l a n d c a r c i n o m a involving intracranial soft tissues.
Acknowledgments T h e surgical p r o c e d u r e s were p e r f o r m e d by Doctors H a r r y S o u t h w i c k and William F. H u g h e s . T h e diagnosis o f m e i b o m i a n g l a n d c a r c i n o m a was m a d e by Dr. J o h n P. Ayer. T h e m a n u s c r i p t was p r e p a r e d by Alberta T h o m a s .
References 1. Ginsburg, J.: Present status of meibomian gland carcinoma. Arch. Ophth., 73:271, 1965. 2. Boniuk, M., and Zimmerman, L. E.: Sebaceous carcinoma
of the eyelid, eyebrow, carunde and orbit. Trans. Am. Acad. Ophth. Otol., 72:619, 1968. 3. Allaire G.: Contribution a l'dtude du polyad6nome ou epith61iome intra-glandulaire. Thesis, Paris, 1891. 4. Thlersch, B.: Der Epithelialkrebs, Namentlich der ~itasseren ltaut, 1865. Cited by Maycda: Das lJdkarcinom. Deutsch. Beitr. Prakt. Augenheilkeit, 56, 1903. 5..Xlagnus, J. A.: Adenocarcinoma of the meibomian gland with secondaries in the liver. Tr. Ophth. Soc. U. King., 67:432, 19-t7. 6. Sweebe, E. C., and Cogan, I). G.: Adenocarcinoma of the meibomian gland. A.M.A. Arch. Ophth., 61:282, 1959. 7. Straatsma, B. R.: Meibomian gland tumors. Arch. Ophth., 56:71, 1956.