CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES SECTION EDITOR: W. RICHARD GREEN, MD blastoma during childhood,1 but livered dose of 5000 rad (50 Gy; 330 Late Malignant Melanoma other multiple ophthalmic malig- rad [3.3 Gy] per fraction) with good After Treatment of nancies in the same patient are a very local tumor control. During follow- Rhabdomyosarcoma of the rare occurrence. We report the case up, the patient developed cataract of a patient who had 2 distinct tu- and strabismus and underwent un- Orbit During Childhood mors involving her left orbit. A child- eventful surgical treatment for cata- A 27-year-old woman developed ma- hood sarcoma was followed 21 years ract. Her final visual acuity was 20/ lignant orbitoconjunctival mela- later by a malignant melanoma. 200 because of macular retinal noma in her left eye 21 years after alterations that were probably sec- treatment of a left orbital embryo- Report of a Case. In 1971, a 6-year- ondary to radiation retinopathy. A nal sarcoma with systemic chemo- old girl developed a tumor in her left surgical procedure to correct stra- therapy and radiation therapy to the orbit. A specimen from an inci- bismus was then performed in 1982, orbit. The coexistence of these 2 ma- sional biopsy of the mass showed and regular follow-up was per- lignancies in the same orbit is very evidence of embryonal sarcoma formed by her ophthalmologist. rare. It may be coincidental, but a ge- (Figure 1A and B). The patient re- In July 1992, at age 27 years, the netic predisposition or late adverse ceived multidrug systemic chemo- patient developed a slowly growing, effects of childhood cancer treat- therapy (including dactinomycin) painless, temporal conjunctival mass ments cannot be excluded. and radiation therapy to the orbit. of the left eye. The patient was then Second malignant tumors can Radiation therapy consisted of an referred to our clinic in September occur in patients treated for retino- exclusive anterior beam with a de- 1992. At that time, she had a slightly A B C D Figure 1. A and B, Biopsy specimen of an orbital lesion, embryonal sarcoma, shows syncytial arrangement of poorly differentiated embryonic mesenchymal cells dispersed in edematous or fibrous stroma (hematein-eosin saffron, original magnification ϫ10 [A] and ϫ40 [B]). C and D, Orbital exenteration because of malignant melanoma shows highly cellular proliferation of fusiform malignant cells with atypical nuclei and prominent nucleoli (hematein-eosin saffron, original magnification ϫ10 [A] and ϫ40 [B]). (REPRINTED) ARCH OPHTHALMOL / VOL 120, AUG 2002 WWW.ARCHOPHTHALMOL.COM 1087 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 pigmented bulbar conjunctival mass logic features and immunopheno- tiva as far as the limbus. The surgical in the temporal area (Figure 2) with type were compatible with the diag- margins were free of tumor. posterior extension on computed to- nosis of malignant amelanotic The patient was then fitted with mography (Figure 3). There were melanoma (tumor cells were posi- transcutaneous implants and a mag- no palpable preauricular or subman- tive for S100 protein and negative for netic retained prosthesis with satis- dibular nodes. Systemic workup vimentin, desmin, actin, myoglo- factory aesthetic results. Regular ruled out metastasis. A biopsy speci- bin, and cytokeratin). The patholo- systemic and ophthalmologic fol- men of the lesion revealed a conjunc- gical characteristics of the exen- low-up was uneventful until Janu- tival malignant melanoma, and the teration specimen confirmed the ary 1999, at which time the patient patient underwent orbital exentera- diagnosis of melanoma (Figure 1C complained of persistent nasal ob- tion in October 1992. Histological ex- and D). The mass was located in the struction. Examination revealed a amination of the biopsy specimen lower conjunctival fornix and ex- mass in the left nasal fossa and max- showed proliferation of malignant tended inferiorly to involve theor- illary sinus (Figure 4). Surgical pleomorphic cells whose morpho- bicularis muscle and bulbar conjunc- treatment consisted of maxillec- tomy with immediate reconstruc- tion with latissimus dorsi free-flap, and pathological examination con- firmed the presence of malignant melanoma (Figure 5A and B). One year later, a sinus relapse was observed on a follow-up com- puted tomographic scan. The pa- tient underwent a salvage opera- tion, and pathological examination confirmed the presence of mela- noma (Figure 5C and D). Dissemi- nated hepatic and bone metastases appeared in August 2000. The pa- tient is currently receiving systemic chemotherapy with interferon and temozolomide. Comment.Theassociationofembryo- nal sarcoma and melanoma in the Figure 2. Slightly pigmented mass adjacent to the temporal region of the left eye. same orbit is a very rare occurrence; Figure 3. Computed tomographic scan of the left orbit shows posterior Figure 4. Computed tomographic image shows recurrent malignant extension of the mass. melanoma involving the orbital apex, left ethmoidal and maxillary sinuses, and nasal fossa. (REPRINTED) ARCH OPHTHALMOL / VOL 120, AUG 2002 WWW.ARCHOPHTHALMOL.COM 1088 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 A B C D Figure 5. A and B, Maxillary recurrence of malignant melanoma shows proliferation of large epithelioid cells (hematein-eosin saffron, original magnification ϫ10 [A] and ϫ40 [B]). C, Sinus relapse of malignant melanoma shows epithelioid-type cells (hematein-eosin saffron, original magnification ϫ20). D, Significant expression of a melanoma-related antigen (HMB 45, ϫ20). to the best of our knowledge, the lit- dren with soft-tissue sarcomas, the in- (rhabdomyosarcoma and malignant erature reports only 1 other case of cidence of second malignant neo- melanoma) occurring in the same pa- orbital melanoma 45 years after suc- plasms was estimated to be 7.5%, but tient at an interval of 20 years; it fur- cessful treatment of rhabdomyosar- these authors reported only 1 case of ther emphasizes the current concern coma.2 The appearance of second melanoma.6 about childhood cancer treatments tumors after treatment of rhabdomyo- The metachronous appearance that might cause severe late effects, es- sarcoma is also a very rare event. In of 2 primary malignancies may be due pecially second cancers. their series describing long-term to several causes. It could be an inci- follow-up of children treated for or- dental occurrence, or it may be related Livia Lumbroso, MD bital rhabdomyosarcoma, Oberlin et to a genetic predisposition. The role Brigitte Sigal-Zafrani, MD al3 did not describe any patient with of previous cancer treatments (che- Thomas Jouffroy, MD a second neoplasm (median follow- motherapy and radiation therapy) Christine Levy, MD up, 8 years). Paulino et al,4 in a smaller cannot be excluded. Jose´ Rodriguez, MD series but with a longer follow-up, de- The possibility of a radioin- Laurence Desjardins, MD scribed a mucoepidermoid carcinoma duced tumor is supported by the pre- Paris, France of the parotid gland occurring at the existing homolateral lesion treated border of the radiation field after ra- by a combination of radiation Corresponding author and reprints: diotherapy of the supraglottic larynx. therapy and chemotherapy; the le- Livia Lumbroso, MD, Institut Curie, An epidermoid carcinoma was de- sion also occurred in the radiation 26 Rue d’Ulm, 75005 Paris, France scribed9yearsafterchemotherapyand field several years after initial treat- (e-mail: [email protected]). radiation therapy for rhabdomyosar- ment. However, the possibility of a coma, and this lesion was attributed genetic predisposition cannot be ex- 1. Abramson DH, Frank CM. Second nonocular 5 tumors in survivors of bilateral retinoblastoma: to radiation therapy. However, the cluded in our patient, despite the ab- a possible age effect on radiation-related risk. various second malignant neoplasms sence of a family history of cancer. Ophthalmology. 1998;105:573-580. described after treatment of rhabdo- This case report describes and 2. Leff SR, Henkind P. Rhabdomyosarcoma and late malignant melanoma of the orbit. Ophthalmol- myosarcomadonotincludemalignant documents the very rare occurrence ogy. 1983;90:1258-1260. melanoma.4,5 In a recent study on chil- of 2 distinct orbital malignancies 3. Oberlin O, Rey A, Anderson J, et al. Treatment (REPRINTED) ARCH OPHTHALMOL / VOL 120, AUG 2002 WWW.ARCHOPHTHALMOL.COM 1089 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 of orbital rhabdomyosarcoma: survival and late angle tumors in the same eye after of systemic malignancy. The tumor effects of treatment results of an international workshop. J Clin Oncol. 2001;19:197-204. treatment. The angle tumors simu- was treated with a radioactive 4. Paulino AC, Simon JH, Zhen W, Wen BC. Long- lated metastases of either the pri- plaque. A week after surgery, 2 light- term effects in children treated with radio- mary choroidal melanoma or an un- gray lesions approximately 1 mm in therapy for head and neck rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 2000;48:1489- recognized systemic malignancy. size were seen at the root of the right 1495. When the eye was removed, we iris at the 7- to 8-o’clock and 10- 5. Raney RB, Anderson JR, Kollath J, et al. Late ef- fects of therapy in 94 patients with localized rhab- found an occult ring melanoma of o’clock positions (Figure 1B). The domyosarcoma of the orbit: report from the In- the ciliary body with invasion of the lesions were observed for 4 months tergroup Rhabdomyosarcoma Study (IRS)–III, angle. but did not enlarge; an iris biopsy 1984-1991. Med Pediatr Oncol. 2000;34:413- 420. was performed and showed spindle 6. Merimsky O, Kollender Y, Issakov J, et al. Mul- Report of a Case. A 71-year-old melanoma cells. Further evalua- tiple primary malignancies in association with soft woman was referred for the evalu- tissue sarcomas.