SMALL CASE SERIES

ity occupying approximately 40% of ultrasonography, the acoustically Cavitary Choroidal the mass (Figure, B). The diagnosis solid mass displayed a hollow cen- Metastasis From Lung was multiple choroidal metastases, tral cavity occupying 20% of the Neuroendocrine Tumor: the largest with cavitation. Systemic mass. (Figure, F) Fine-needle bi- Report of 3 Cases evaluation revealed biopsy-proven opsy disclosed pleomorphic cells primary bronchial NET with clini- more consistent with melanoma Neuroendocrine tumors (NET) arise cal features of carcinoid syndrome. than metastasis. Therapeutic op- from the diffuse neuroendocrine sys- Plaque radiotherapy was per- tions included radiotherapy or tem in the gastrointestinal tract, formed. Thirteen months after enucleation, and the patient pre- bronchopulmonary system, and the therapy, the patient died of sys- ferred enucleation of the left eye. urogenital tract. Generically these tu- temic complications of metastatic Gross examination of the enucle- mors have been referred to as car- NET. ated eye showed a tan choroidal cinoid tumors, but more recent sci- Case 2. A 77-year-old woman mass with overlying shallow reti- entific evaluation introduced by the with breast cancer developed blurred nal detachment. Microscopic analy- vision in the right eye and was found sis of the lesion (previously de- World Health Organization has re- 5 classified them as NET based on size, to have an amelanotic choroidal scribed ) showed a malignant proliferative rate, localization, dif- mass, presumed to represent breast neoplasm with pigmented dendriti- ferentiation, and hormone produc- cancer metastasis. Her visual acu- form cells with slender processes and ity was 20/50 OD and 20/20 OS. The a fluid-filled, intralesional cavity tion. Neuroendocrine tumors can be ϫ ϫ a low-grade malignancy (carcinoid mass measured 12 12 7mmand without an endothelial lining. There tumor)1 or a more aggressive vari- showed associated subretinal fluid was no area of necrosis, hemor- ant. This tumor rarely metastasizes (Figure, C). B-scan ultrasonogra- rhage, or inflammation surround- to the ocular region. However, when phy revealed an echogenic mass with ing the cystic cavity. There was no this does occur, gastrointestinal NET an acoustically hollow cavity occu- sign of trauma from the needle bi- typically spreads to the orbit, pying 30% of the mass (Figure, D). opsy inducing the cavity. Immuno- whereas bronchial NET classically Fine-needle aspiration biopsy re- histochemical staining of the mass metastasizes to the choroid.2 vealed an NET with morphology and showed immunoreactivity for cyto- Ocular ultrasonography is im- immunocytochemical staining (posi- keratin markers (CAM 5.2, AE1), portant in differentiating carcinoid tive AE1/AE3, EMA, chromogra- calcitonin, chromogranin, and syn- metastasis from amelanotic mela- nin, synaptophysin and negative aptophysin, features that were in- noma in that metastasis is usually HMB45, Melan A, CD45) sugges- dicative of a metastatic NET. Re- echogenic while melanoma is typi- tive of carcinoid tumor. Systemic sults of immunoreactivity testing for cally echolucent.3,4 Occasionally, evaluation found a primary tumor in melanoma marker HMB45, as well melanoma can also show intrale- the lung and possible liver metasta- as S100, vimentin, and breast carci- sional cavitation, a feature not pre- ses. Plaque radiotherapy was per- noma markers were negative. Sys- viously described with metastases. formed, and the tumor regressed to temic evaluation revealed a lung In this article, we describe a new ob- 2.6 mm on the last follow-up visit 4 nodule on the right middle lobe with servation of cavitation within biopsy- months later. The liver neuroendo- positive immunoreactivity to AE1, crine metastases were later con- calcitonin, chromogranin, and syn- proven NET (carcinoid) metastasis 5 in 3 cases. firmed by biopsy and the patient aptophysin, consistent with NET. started receiving chemotherapy. On the last follow-up visit at 22 Report of Cases. Case 1. A 59-year- Case 3. A 77-year old woman with months, her visual acuity remained old, asymptomatic, healthy woman a 4-year history of nonproductive unchanged in the right eye and the was found to have an amelanotic cho- cough had a 4-month history of pro- choroidal metastases were stable. roidal mass in her left eye on rou- gressive blurred vision in the left eye. tine examination. Visual acuity mea- Her visual acuity was 20/20 OD, and Comment. Cavitation of an intra- sured 20/20 OU. The mass measured hand motions OS. Fundus exami- ocular tumor has been documented 4ϫ4 ϫ 2 mm. Ocular ultrasound nation showed 2 subtle, amela- with uveal melanoma, ciliary body showed medium acoustic hollow- notic, acoustically solid choroidal melanocytoma, and retinoblas- ness and medium internal reflectiv- masses on the right eye located along toma.6-12 Cavitary melanoma can ity. The lesion remained stable for 4 the inferotemporal arcade measur- show unifocal or multifocal cavi- years but then showed growth to ing 3ϫ1.5ϫ1.5 mm and 1ϫ1 ϫ ties, some of which occupy up to 90% 16ϫ15ϫ8 mm. (Figure,A)Two 0.5 mm with no associated subreti- of the tumor.8 The origin of intratu- new, smaller tumors were also noted. nal fluid. In the left eye, there was a moral cavities is speculated to be the On ocular ultrasonography, the main solitary amelanotic mass measur- result of tumor necrosis, intrale- lesion was acoustically solid, with a ing 13ϫ12ϫ6.4 mm with serous sional hemorrhage or accumulation well-defined, acoustically hollow cav- retinal detachment. (Figure, E) On of mucoproteinaceous substance.7,8

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 In this study, we found cavitation A B within 3 choroidal metastases, all of which proved to be large NET from primary site in the lung. Based on this finding, patients who present with a cavitary amelanotic choroidal mass should have systemic evaluation for a primary cancer and possible con- firmation with fine-needle aspira- tion biopsy of the intraocular tumor, as large choroidal metastases as well C D as melanoma can show cavitation. Carol L. Shields, MD Emil Anthony T. Say, MD Natalie A. Stanciu, MD Carlos Bianciotto, MD Carl J. Danzig, MD Jerry A. Shields, MD Author Affiliations: Oncology Ser- E F vice, Wills Eye Institute, Thomas Jef- ferson University, Philadelphia, Pennsylvania. Correspondence: Dr C. L. Shields, Oncology Service, Wills Eye Hospi- tal, 840 Walnut St, Ste 1440, Phila- delphia, PA 19107 (carol.shields @shieldsoncology.com). Financial Disclosure: None re- ported. G H Funding/Support: This study was supported in part by a donation from Michael, Bruce, and Ellen Ratner, New York, New York (Drs J. A. Shields and C. L. Shields); Mellon Charitable Giving from the Martha W. Rogers Charitable Trust (Dr C. L. Shields); the Rosenthal Award of the Macula Society (Dr C. L. Figure. Neuroendocrine tumor (NET) metastasis to the choroid demonstrating cavitation. A and B, A Shields); the Paul Kayser Interna- 59-year-old woman with choroidal metastasis from lung NET was found to have a large amelanotic tional Award of Merit in Retina Re- choroidal metastasis (A) with surrounding subretinal fluid. B-scan ultrasonography (B) showed a search, Houston, Texas (Dr J. A. dome-shaped echodense choroidal mass of 7.8 mm thickness with central cavitation occupying approximately 40% of the mass. C and D, A 77-year-old woman with choroidal metastasis from lung NET Shields); and the Eye Tumor Re- was found to have a large amelanotic choroidal metastasis (C), shown on fine-needle aspiration biopsy. search Foundation, Philadelphia, B-scan ultrasonography (D) showed an echogenic dome-shaped choroidal mass with central cavitation, Pennsylvania (Drs J. A. Shields and occupying approximately 30% of the mass. E and F, A 77-year-old woman with bilateral multifocal metastasis from lung NET tumor demonstrated a large amelanotic choroidal metastasis (E) with C. L. Shields). cavitation on B-scan ultrasonography (F) occupying approximately 20% of the mass. G and H, Following enucleation, microscopic analysis of case 3 revealed a fluid-filled cavity within the tumor without 1. Jensen RT, Doherty GM. Carcinoid tumors and surrounding inflammation or necrosis seen on low power (G; hematoxylin-eosin; original magnification the carcinoid syndrome. In: DeVita VT Jr, Hell- ϫ15) and without a continuous endothelial lining on higher magnification (H; hematoxylin-eosin; original man S, Rosenberg SA, eds. Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, magnification ϫ250). PA: Lippincott Williams & Wilkins; 2005:1559- 1574. 2-4 2. Shields JA, Shields CL. Metastatic tumors to the With retinoblastoma, intratumoral nography. It is well established that uvea, retina, and optic disc. In: Shields JA, cavities are suggestive of well- most metastatic tumors appear ech- Shields CL, eds. Intraocular Tumors: An Atlas differentiated retinoblastoma that odense on B-scan and show medium and Textbook. Philadelphia, PA: Lippincott Wil- liams & Wilkins; 2008:198-227. show minimal response to chemo- to high internal reflectivity on A- 3. Harbour JW, De Potter P, Shields CL, Shields JA. therapy and radiotherapy.6 scan ultrasonography.2-4 Melanoma Uveal metastasis from carcinoid tumor: clinical observations in nine cases. Ophthalmology. 1994; The differentiation of a choroidal tends to be echolucent on B-scan and 101(6):1084-1090. metastasis from amelanotic mela- show low internal reflectivity on A- 4. Shields CL, Shields JA, Gross NE, Schwartz GP, noma depends on several clinical fea- scan ultrasonography.2-4 Until now, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology. 1997;104(8):1265- tures as well as diagnostic testing with cavitation within a choroidal lesion 1276. fluorescein angiography and ultraso- was most suggestive of a melanoma. 5. Eagle RC Jr, Ehya H, Shields JA, Shields CL.

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©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 Choroidal metastasis as the initial manifesta- External examination (B.N.W.) tion of a pigmented neuroendocrine tumor. Arch A Ophthalmol. 2000;118(6):841-845. revealed an approximately 1-cm 6. Mashayekhi A, Shields CL, Eagle RC Jr, Shields mass just inferior and lateral to the JA. Cavitary changes in retinoblastoma: rela- left medial canthus (Figure 1A). tionship to chemoresistance. Ophthalmology. 2005;112(6):1145-1150. The overlying skin was erythema- 7. Lois N, Shields CL, Shields JA, Eagle RC Jr, De tous and the lesion firm. The right Potter P. Cavitary melanoma of the ciliary body: a study of eight cases. Ophthalmology. 1998; medial canthus was minimally ery- 105(6):1091-1098. thematous, but without mass. The B 8. Zakka KA, Foos RY, Spencer WH, Kerman BM, remaining anterior segment and reti- Newman NM, Pettit TH. Cavitation in intra- ocular malignant melanoma. Arch Ophthalmol. nal examinations were normal 1982;100(1):112-114. bilaterally. 9. Croxatto JO, Malbran ES, Lombardi AA. Cavi- The patient underwent a left na- tary melanocytoma of the ciliary body. Ophthalmologica. 1984;189(3):130-134. solacrimal probing and irrigation in 10. Capea´nsC,Pin˜ eiro A, Blanco MJ, et al. Ultrasound the office with topical anesthetic. biomicroscopic findings in a cavitary melanocy- Figure 1. Case 1. A, Newborn female with initial toma of the ciliary body. Can J Ophthalmol. 2003; Copious yellow-white material was presentation of a left dacryocele with minimal 38(6):501-503. expressed via the lower puncta when redness on the right. B, Sequential right 11. Becerra EM, Saornil MA, Blanco G, Me´ndez MC, the No. 0 Bowman probe was passed dacryocele days later, after successful probing Muin˜ os Y, Esteban MR. Cavitary choroidal and irrigation of the left lacrimal system. melanoma. Can J Ophthalmol. 2005;40(5): into the lacrimal sac and then into 619-622. the nasolacrimal duct. No attempt 12. Faraj H, Levy-Gabriel C, Lumbroso-Le Rouic L, was made to accomplish intranasal “yellow pus” draining from the left et al. Cavitary choroidal melanoma in a child [in French]. J Fr Ophtalmol. 2006;29(5):559-563. “metal-on metal” contact. The lac- eye since 4 days of age, which then rimal system was irrigated with stopped at 10 days of age. Subse- normal saline. The patient was quently, the left lower eyelid swell- prescribed moxifloxacin and tobra- ing became firm and developed sur- mycin/dexamethasone drops, ad- rounding erythema. Oral amoxicillin Sequential Bilateral ministered each alternating hour was prescribed by the pediatrician. Dacryocele while awake. The parents were in- On initial examination by Ophthal- structed to perform nasolacrimal sac mology (B.M.S.) at 12 days old, a Dacryoceles, also known as lacri- massage. firm, erythematous, cystlike lesion mal sac mucocele, amniocele, am- One day later, the parents re- inferior and lateral to the medial can- niotocele, or dacryocystocele, are a ported discharge from both eyes. thus, causing an upward distortion rare form of congenital nasolacri- Massage and eye drop administra- of the eyelid margin, (Figure 3A) mal duct obstruction with both tion were continued. Five days later, consistent with an infected dacryo- proximal and distal obstruction re- examination revealed a new right cele was seen. sulting in lacrimal sac enlarge- dacryocele without infection but Attempted decompression by ment. Usually presenting at birth, with persistent discharge bilater- moderate pressure failed. The lacri- there is significant risk for infec- ally (Figure 1B). The child was afe- mal system was probed into the lac- tion. Probing and excision of an as- brile and feeding well. Office right rimal sac without passing the probe sociated intranasal cyst under gen- nasolacrimal probing and irriga- down the nasolacrimal duct. Copi- eral anesthesia may be needed. We tion was performed, again produc- ous greenish-yellowish material present 2 infants who underwent ing copious discharge. One day later, drained. The parents were in- probing for a unilateral dacryocele the right dacryocele had recurred. structed to continue lacrimal sac who subsequently developed a dac- Gentle massage failed to decom- compression and oral antibiotics. ryocele on the unaffected side. We press the dacryocele. Repeated office Two days later, the dacryocele was suggest that if a unilateral dacryo- probing and irrigation was per- decreased in size and the erythema cele is probed under general anes- formed. The next day, the dacryo- resolved. Compression by the oph- thesia it may be prudent to evalu- cele returned. There was persistent thalmologist yielded approxi- ate the contralateral side, even if discharge from the left eye, despite mately 2 to 3 mL of discharge and asymptomatic. Probing and exci- apparent resolution of the left the dacryocele decompressed. Sub- sion of an intranasal cyst, if present dacryocele. sequently, the dacryocele recurred. on the asymptomatic side, may pre- The patient was hospitalized, At 29 days old, the patient un- vent development of a dacryocele where probing and irrigation were derwent left nasolacrimal probing and a second anesthesia. performed under general anesthe- and intranasal cyst excision with in- sia. Intranasal endoscopy revealed tranasal endoscopy under general Report of Cases. Case 1. An other- bilateral intranasal cysts (Figure 2), anesthesia for a persistent dacryo- wise well, full-term, 6-day-old girl which were excised. All symptoms cele. Immediately after probing and was referred for evaluation of a were resolved on follow-up exami- cyst excision, the dacryocele re- purple mass present since birth near nation 1 month later. solved and did not recur. the left medial canthus. The par- Case 2. An otherwise healthy, full- At 40 days old, discharge was first ents noted yellowish discharge from term, 2-day-old baby was observed noted from the right eye. At 61 days both eyes. She was feeding well with- to have swelling of the left lower eye- old, a firm swelling was found just out dyspnea. lid medially. The parents reported inferolateral to the medial canthus

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