Orbital metastasis of choriocarcinoma from a testicular malignant germ cell tumor: A late unfavorable prognostic sign

Hayyam Kiratli; Abdullah Ağın; İrem Koç; Orkun Akman; Figen Söylemezoğlu

doi:10.4103/IJO.IJO_2771_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 524-525

Orbital metastasis of choriocarcinoma from a testicular malignant germ cell tumor: A late unfavorable prognostic sign

Hayyam Kiratli¹, Abdullah Ağın², İrem Koç¹, Orkun Akman³, Figen Söylemezoğlu⁴
¹ Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey
² Department of Ophthalmology, University of Health Science, Haseki Training and Research Hospital, Istanbul, Turkey
³ Department of Pathology, Yuksek Ihtisas University School of Medicine, Ankara, Turkey
⁴ Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey İstanbul, Turkey

Date of Submission	22-Oct-2022
Date of Acceptance	24-Jan-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Hayyam Kiratli
Professor of Ophthalmology, Ocular Oncology Service, Hacettepe University School of Medicine, Sihhiye - 06100, Ankara, Turkey İstanbul
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2771_22

Abstract

A 43-year-old man presented with a painful left proptosis 6 years after he had had orchiectomy for a mixed germ cell tumor. He also previously developed pulmonary and brain metastases, which were treated with chemotherapy and radiotherapy. Magnetic resonance imaging studies revealed an irregular mass confined to the left superior orbit. The neoplastic cells stained positive for human placental lactogen and sal-like protein 4 but negative for Alpha fetoprotein (AFP), CD30, and octamer binding transcription factor 3/4, compatible with metastatic choriocarcinoma. A painful mass located in the superior orbit in a young man must arouse the suspicion for a metastatic testicular carcinoma.

Keywords: Choriocarcinoma, metastasis, mixed germ cell tumor, orbit, radiotherapy, testis

How to cite this article:
Kiratli H, Ağın A, Koç &, Akman O, Söylemezoğlu F. Orbital metastasis of choriocarcinoma from a testicular malignant germ cell tumor: A late unfavorable prognostic sign. Indian J Ophthalmol Case Rep 2023;3:524-5

How to cite this URL:
Kiratli H, Ağın A, Koç &, Akman O, Söylemezoğlu F. Orbital metastasis of choriocarcinoma from a testicular malignant germ cell tumor: A late unfavorable prognostic sign. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 6];3:524-5. Available from: https://www.ijoreports.in/text.asp?2023/3/2/524/374988

Orbital metastatic tumors account for 1%–13% of all orbital neoplastic lesions and may be encountered in 2%–5% of cancer patients.^[1] Breast carcinoma is the leading cause of orbital metastasis seen in 36.3% of cases, followed by melanoma and prostate cancer in 10.1% and 8.5% of cases, respectively.^[1] The orbit is rarely involved by metastatic testicular cancer, and only a few well-documented biopsy-proven cases have been reported so far.^{[2],[3],[4],[5],[6]}

Herein, we present the rare case of a patient with a testicular malignant mixed germ cell tumor that metastasized to the orbit as choriocarcinoma. The study adhered to the tenets of the Declaration of Helsinki.

Case Report

In 2016, a 43-year-old man presented with left-sided proptosis that developed within 3 months. Initially, he had intermittent pain, which became permanent and severe more recently. His medical history included a radical orchiectomy followed by chemotherapy for a testicular malignant germ cell tumor in 2010. Three years later, he developed pulmonary metastases, which were treated with lobectomies and chemotherapy. In 2015, he was found to have a frontal lobe metastasis, which was surgically removed and then irradiated. On our ocular examination, his best corrected visual acuity was 20/20 in each eye. Left eye proptosis with slight inferior dystopia of 2 mm was found. There was mild left upper eyelid ptosis. Hertel measurements were 16 mm right eye and 18 mm left eye. The patient had difficulty in upward gaze, indicating the presence of −1 ocular motility restriction in the superior rectus. The rest of the examination of both eyes was normal. Orbital magnetic resonance imaging (MRI) studies revealed an irregular mass in the superior part of the left orbit [Figure 1]. A mild heterogenous enhancement was observed following contrast agent administration.

Figure 1: T1-weighted orbital MRI. (a) Coronal view showing an irregular mass in the left superior orbit (arrows) without bone destruction. (b) Sagittal section of the left orbit demonstrates the inferior displacement of the superior rectus muscle (arrows) by the tumor. * Indicates the previously treated frontal lobe mass. MRI = magnetic resonance imaging

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An incisional biopsy was performed through a sub-brow approach. Histopathologic evaluation of the specimen disclosed that the tumor was composed of mononuclear cells with clear eosinophilic cytoplasm and multinucleated pleomorphic cells with large eosinophilic cytoplasm [Figure 2]a. Immunohistochemical studies showed that the neoplastic cells stained positive for human placental lactogen (HPL) [Figure 2]b and sal-like protein 4 (SALL4) but negative for AFP, CD30, and octamer binding transcription factor 3/4 (OCT 3/4). Multinucleated giant cells stained positive for inhibin also. These findings favored the diagnosis of metastatic testicular choriocarcinoma.

Figure 2: Histopathologic and immunohistochemical studies showed (a) the tumor was composed of infiltrative mononuclear cells with clear eosinophilic cytoplasm and multinucleated pleomorphic cells (H and E, original magnification ×40) and (b) positive staining for HPL (original magnification ×20). H and E = hematoxylin and eosin, HPL = human placental lactogen

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The patient received 24 Gy orbital radiotherapy, but the tumor continued to grow. In March 2017, etoposide and carboplatin regimen was instituted, but he expired because of severe neutropenia, thrombocytopenia, and ensuing septic emboli.

Discussion

Testicular germ cell tumors are the most common solid malignant tumors in young men.^[7] Classification and histopathologic evaluation of testicular tumors are challenging and continue to evolve rapidly. Based on 2016 World Health Organization (WHO) classification and its 2022 update, our patient had type II tumor, which is linked to germ cell neoplasia in situ (GCNIS) and seen in postpubertal age.^[7],[8] Tumors of a single histologic type may derive from GCNIS, and these are further classified as seminomatous and non-seminomatous types.^[7] Choriocarcinoma, now considered as a subtype of non-seminomatous testicular tumor, is a biphasic malignancy containing syncytiotrophoblasts and cytotrophoblasts.^[7] While 8% of testicular germ cell tumors may contain a choriocarcinomatous component, pure choriocarcinoma accounts for only 0.3% of all testicular germ cell tumors.^[9] This is an aggressive tumor with a significant proclivity for angioinvasion; hence, early metastases to lungs, liver, gastrointestinal tract, brain, and bones are common.^[7],[9]

Ocular and orbital metastases of testicular choriocarcinoma are, however, exceedingly rare. A few cases of choroidal and scleral metastases have been reported.^[9],[10] We were able to find only one well-documented orbital case, in which a 34-year-old man with superior rectus complex involvement was described.^[3] Other reported patients with orbital metastatic testicular cancers mostly had seminoma, which is a distinctly different testicular malignancy spreading through lymphatics in contrast to choriocarcinoma which metastasizes via hematogenous route.^[2],[5],[6] Interestingly, however, seminomas also metastasized to the superior parts of the orbit and produced painful proptosis.^[2],[5],[6] Due to the rarity of this condition, there is no consensus over the best treatment; but based on the literature review done by Dutton and Proia^[2] and on more recently reported cases,^[3],[4] most patients were managed by incisional biopsy followed either by chemotherapy or radiotherapy or by corticosteroid administration. Patients with orbital metastatic seminoma had a relatively good prognosis.^[2],[5],[6] In contrast, our patient and the one reported by Sivagnanasithiyar et al.^[3] suggest that choriocarcinoma metastatic to the orbit appears to portend a dismal prognosis.

Conclusion

Although orbital masses are associated with many conditions, including primary and secondary, the characteristics of the primary tumor are the main factor affecting survival in metastatic cases. Metastatic testicular carcinoma must be considered in the differential diagnosis of a painful mass located in the superior parts of the orbit, particularly in young men.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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