Although Testicular Germ Cell Carcinoma Is Rare

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immediately referred to an oncologist for further meta- Improved Systemic Chemotherapy static workup. The patient was diagnosed with meta- for Metastatic Testicular Choriocarcinoma static testicular choriocarcinoma requiring radical or- Can Result in Excellent Prognosis chiectomy followed by a 5-cycle chemotherapy regimen for Life and Vision of bleomycin sulfate, etoposide, and cisplatin. The patient responded well to the chemotherapy sys- temically, and serial ultrasonography examinations lthough testicular germ cell carcinoma is rare, showed a dramatic decrease in the thickness of the cho- it is the most common malignant neoplasm in roidal mass from 12.4 mm to 3.0 mm. Three months af- A men between the ages of 15 and 35 years. We ter chemotherapy, the patient was clinically in remis- report a case of metastatic choriocarcinoma involving the sion. The choroidal mass continued to decrease in size choroid that was effectively treated with chemotherapy until it became undetectable by ultrasonography and had an excellent visual outcome. (Figure 1) and the left afferent pupillary defect re- solved. However, the visual acuity was 6/200 OS from a Report of a Case. A 23-year-old healthy man visited the nonclearing vitreous hemorrhage. Five months into re- emergency department with decreased vision in his left mission without resolution of the hemorrhage, the pa- eye. His best-corrected visual acuities were 20/20 OD and tient underwent a 25-gauge pars plana vitrectomy of the light perception OS. There was a left afferent pupillary left eye. He later developed an epiretinal membrane and defect. Slitlamp and fundus examination results of the cataract that required a vitrectomy with membrane peel- right eye were unremarkable. Dilated funduscopic ex- ing and cataract extraction. Three years after his initial amination of the left eye was limited due to a vitreous diagnosis, the patient remains in remission with a visual hemorrhage. Standardized A-scan and contact B-scan ul- acuity of 20/40 OS. Subretinal fibrosis has replaced the trasonography of the left eye showed a large, irregularly previously active metastatic lesions (Figure 2). shaped and structured, highly vascular mass lesion in the superotemporal quadrant (Figure 1). The mass mea- Comment. Choriocarcinoma is an extremely rare cause sured approximately 19.0ϫ15.8 mm in diameter with a of choroidal metastasis. A recent meta-analysis1 re- maximal thickness of 12.4 mm. ported 15 cases of testicular choriocarcinoma to the cho- On further detailed review of systems, the patient ad- roid. Seventy-three percent of these patients (11 of 15 mitted to a 6-week history of testicular pain. An initial patients) had a poor prognosis, with survival of less than workup revealed a testicular mass, and chest radiogra- 2 months in the majority of these cases. Most of these phy revealed 2 mass lesions in the lungs. The patient was reports with poor survival were prior to the 1970s. Ad-

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Figure 1. Ultrasonography. A, Contact B-scan at the initial visit showing the irregular shape and irregular structure of the solid mass lesion noted superotemporally (arrows). B, Standardized A-scan showing the irregular reflectivity (arrows). Marked vascularity was noted during the dynamic portion of the examination. C, Transverse B-scan 7 months following treatment showing vitreous hemorrhage (VH), posterior VH vitreous detachment, and a small area of thickening in the fundus where the tumor was previously located before treatment (arrow).

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©2008 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Figure 2. Fundus photographs. In the periphery, fundus photographs show retinal pigment epithelial changes with subretinal fibrosis in the area of previously active metastatic lesions.

vances in chemotherapy involving cisplatin-based regi- 4. Zech JC, Subiger L, Chiquet C, Bouvier R, Trepsat C. Testicular choriocarci- mens markedly improved the prognosis in the mid- noma metastatic to the choroid. Retina. 1999;19(2):164-165. 1970s. In fact, germ cell tumors have become one of the most curable solid neoplasms, with survival rates of approximately 80% for advanced disease and nearly 100% Perivascular Epithelioid Cell Tumor for early disease.2 The visual prognosis in metastatic choriocarcinoma of the Orbit to the choroid has been poor. Most patients described prior to the 1970s died without any improvement in their erivascular epithelioid cell tumors (PEComas) are vision. In the 2 cured patients, one’s visual acuity was rare mesenchymal neoplasms comprising angio- not reported,3 whereas the other’s was 20/1000 due to myolipoma, lymphangiomyomatosis, and clear P 1 scarring from the regressed tumor.4 Despite the relative cell “sugar” tumor of the lung. In 1992, the term PEC afferent pupillary defect and the large tumor mass (12.4 was introduced to characterize distinct HMB45-positive 2 mm), our case had an excellent visual outcome of 20/ cells that seemed to originate from blood vessel walls. 2 40, highlighting the importance of close and continued Zamboni et al coined the term PEComa to describe a pan- ophthalmic care. During chemotherapy, serial ultraso- creatic clear cell sugar tumor lesion indistinguishable from nography examinations were essential in showing the lung clear cell sugar tumor. Recently, PEComas have been treatment response by documenting the decrease in tu- recognized at various sites, most often in middle-aged fe- 3 mor size. Ophthalmologists must be patient with the che- male patients. PEComas are characterized by typical chro- motherapy before considering surgical therapy, al- mosomal imbalances, suggesting PECs as distinct tu- 4 though surgical options should still be considered after mor cells. treatment if appropriate. Close follow-up in conjunc- A single case of orbital PEComa has been reported in 5 tion with an oncologist is required. a 9-year old child. Here we report a PEComa in the orbit of a 54-year-old patient. Rahul N. Khurana, MD Cathy DiBernardo, RDMS Report of a Case. A 54-year-old male patient had a slowly James T. Handa, MD progressing, painless swelling of the right temporal lower eyelid (Figure, A). Ophthalmological examination re- Correspondence: Dr Handa, Cancer Research Bldg 2, sults of the healthy patient were otherwise normal. Or- Room 144, 1550 Orleans St, Baltimore, MD 21287- bital examination showed a soft fluctuant mass in the an- 9277 ([email protected]). terior inferotemporal orbit without overlying cutaneous Financial Disclosure: None reported. changes. Orbital ultrasonography and computed tomog- raphy revealed a highly reflective, demarcated, hyper- 1. Kavanagh MC, Pakala SR, Hollander DA, O’Brien JM. Choriocarcinoma metastatic to the choroid. Br J Ophthalmol. 2006;90(5):650-652. dense, contrast-enhancing, round lesion measuring 2. Oh WK. Overview of treatment for testicular germ cell tumors. In: Rose B, 1.5ϫ1.0ϫ1.0 cm. The tumor with large feeder vessels ed. UpToDate. Waltham, MA: UpToDate; 2007. was completely excised through an anterior orbitotomy 3. Nakajima H, Oki M, Matsukura S, Nakamura M, Tokunaga M, Ando K. Germ cell tumors: case 1, intraocular metastases from testicular cancer. J Clin Oncol. with subciliary incision. No recurrence was detected dur- 2004;22(9):1753-1755. ing 17 months of follow-up.

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