Immature teratoma of the ovary: A Pandora’s box-an oncologist’s perspective

INTRODUCTION

Affecting the postmenopausal population, a mature cystic teratoma typically harbors squamous cell carcinoma as the most common component.^[1] However, other elements of differentiation have been previously reported.^[2] Evidence-based management is the motto of oncology, even for the rarest malignancies. Therefore, it is prudent to discuss the dilemma of immature teratoma that affects the adolescent and young adult age group – a nation’s future and, in our case, our patient.

In this case report, we focused on bringing a more comprehensive and practical approach to managing a case of immature teratoma with oligodendroglial differentiation in a young lady diagnosed in the 2^nd decade of her life. The Institutional Review Board approved this study, and the patient’s consent was obtained.

CASE REPORT

A 22-year-old unmarried, nulliparous female presented to our center in post-operative status with the diagnosis of an immature teratoma. The patient had initially presented with abdominal distension and pain in the lower abdomen ×3 months. Baseline tumor markers were normal. Contrast-enhanced computed tomography of the abdomen had depicted a large cystic mass in the abdominopelvic region ~17 cm, arising from the left ovary. She had undergone exploratory laparotomy and left salpingo-oophorectomy in May 2023. The post-operative histopathology report (HPR) suggested a Grade-3 left ovarian immature teratoma of size ~15 cm, with oligodendroglial differentiation; ovarian surface and fallopian tube were free of deposits – pT1a (IA), [Figure 1a-e]. After a thorough discussion in the multidisciplinary tumor board (MDT) – National Comprehensive Cancer Network guidelines were discussed after a brief review of the literature; given Grade-3 disease, the board decided to proceed with three cycles of BEP (Inj. Bleomycin 30 IU on days – 1,8,15, Inj. Etoposide @ 100 mg/m²/day × 5 days, Inj. Cisplatin @20 mg/m² day × 5 days) every 21 days, followed by one cycle of EP as per protocol. Post-operative imaging suggested no residual/recurrent disease with normal tumor markers. Before treatment initiation, the option of fertility preservation was discussed; however, the patient and her respondents were unwilling and signed a written consent. The patient completed three cycles of BEP. Post-3^rd cycle, she was admitted for febrile neutropenia with Grade-2 diarrhea and was managed symptomatically with i/v antibiotics, antidiarrheal measures, and supportive care. She completed cycle 4 with EP (25% dose reduction) on September 27, 2023. Tumor markers were normal post-chemotherapy completion. The case was re-discussed in MDT, and the patient was planned to be kept on 3-monthly follow-up for the first 2 years – monitoring with ultrasonography whole abdomen and pelvis and serum tumor markers and then every 4^th–6^th monthly till the 5^th year.

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Figure 1:

Left salpingo-oophorectomy specimen: (a) Focal areas of moderately cellular neoplasm composed of round to oval tumor cells with mild pleomorphic vesicular nuclei; (b) scanty to moderate cytoplasm and perinuclear halo arranged in sheets and singles against a glio-fibrillary background; and (c) intervening stroma with frequent thin-walled branching blood vessels; (d and e). No significant mitosis, IMCT Grade-3. (200X; Hematoxylin and eosin stain).

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The patient has a disease-free survival of 9 months at the time of writing this paper. The last follow-up was in May 2024, and the previous telephonic update was in June 2024.

DISCUSSION

Squamous cell carcinoma is the most common malignancy arising in a mature cystic teratoma; it tends to affect the postmenopausal population.^[1] Other elements of differentiation, including but not limited to gastrointestinal adenocarcinoma, sarcoma, and melanoma, have also been reported as part of various case reports.^[2]

The presentation of our patient at the age of 22 years with abdominal pain corresponds to the previously reported young adult age group and chief complaint;^[1-5] however, its diagnosis in the adolescent age group has also been documented.^[6]

As part of primary treatment, our patient underwent exploratory laparotomy with the left salpingo-oophorectomy. On HPR, it was found to be a cystic ovary ~15 cm tumor size in largest dimension, Grade-3, with the typical histopathological finding of round to oval tumor cells having mild pleomorphic vesicular nuclei, scanty to moderate cytoplasm and perinuclear halo (fried egg appearance), arranged in sheets and singles against a glial fibrillary background, and frequent thin-walled branching blood vessels. All the microscopic findings correspond to previously published reports.^[1-6] The patient tolerated three cycles of BEP and one cycle of EP with a manageable toxicity profile and was disease-free for 9 months; however, previously reported cases received systemic therapy at the time of relapse and succumbed to the disease eventually.^[4] A table depicting the relevant findings from the present case report with other reported cases and a case series has been summarized below for the readers [Table 1].

Despite our report being limited to a single patient’s data, we cannot undermine that it helped us to understand the dictum “the eyes don’t see what the mind doesn’t know” – this rarest form of presentation of immature teratoma with oligodendroglial differentiation. An early surgical intervention followed by systemic therapy with BEP, in this case of Stage-IA Grade-3, is prudent to prevent relapse and or recurrence for better survival outcomes. This entity is sensitive because this is the age group of women that constitutes the fertile population and thereby affects all aspects of health. Ultimately, the goal is to help achieve an overall normal life expectancy, a good quality of life, and fertility preservation through oocyte banking and early surgical intervention, as most cases are localized to a single ovary.

CONCLUSION

Our case highlights the importance of rigorous histological and immunohistochemical examination in patients with immature teratoma. As rare as it may be, such specimens can harbor neural tissue elements, including the rarest of them all, an oligodendroglioma. These patients should be promptly treated based on the stage and grade of the mature/immature teratomas as per institutional guidelines since neural elements, especially oligodendroglial components, are a harbinger of worse prognosis and should be nipped at its bud to improve the survival outcomes.