A rare presentation of malignant mesothelioma of the tunica vaginalis managed with immunotherapy and review of the literature

Our plain-English summary of A rare presentation of malignant mesothelioma of the tunica vaginalis managed with immunotherapy and review of the literature, by K. Mishra, S. Siddiquee, A.R. Mislang (Clinical Case Reports, 2023).

The study

• Authors: K. Mishra, S. Siddiquee, A.R. Mislang
• Journal: Clinical Case Reports
• Published: 2023;11(6):e7610
• Type of article: Case report
• Cited by: 8 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Key Clinical Message: We describe the first case in literature of malignant mesothelioma of the tunica vaginalis that has shown partial response to systemic immunotherapy (ipilimumab-nivolumab) post orchiectomy, warranting further investigation in a trial setting.

Abstract: We present a case report of an 80-year-old ex-smoker with a rare diagnosis of metastatic mesothelioma of the tunica vaginalis, managed with immunotherapy. The patient, with no known history of asbestos exposure, presented with a left scrotal mass and pain. Scrotal ultrasound confirmed a large paratesticular mass, and computed tomography (CT) of the chest, abdomen, and pelvis revealed a bilobed mass in the left scrotal compartment without associated inguinal or abdominopelvic lymphadenopathy, and an indeterminate, subcentimeter, bi-basal subpleural nodules. He underwent a left orchiectomy, and histopathology confirmed the diagnosis of a paratesticular mesothelioma. Postoperatively, the patient had a positron emission tomography (PET) scan showing a new right pleural effusion as well as increasing size of the lobar and pleural nodules bilaterally, all metabolically active and suggestive of progressive metastatic disease. The patient was commenced on ipilimumab and nivolumab immunotherapy, a regimen indicated for malignant pleural mesothelioma; however, the efficacy on paratesticular mesothelioma is not known. After 6 months of treatment, the patient demonstrated a partial response to immunotherapy, with a reduction in the size of known pleural nodules and effusion.Literature review suggests that diagnosis requires a high index of suspicion and patients commonly have metastatic disease at the time of diagnosis. Orchiectomy is a common management modality. However, the role, regimen, and benefits of systemic therapy are unclear, warranting further studies investigating management strategies.

© 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.

In plain English

In this case report with a review of published cases, the authors described an 80-year-old man, an ex-smoker with no known asbestos exposure, who had a left scrotal mass and pain. After his left testicle was removed, the tumor was diagnosed as malignant epithelioid mesothelioma of the tunica vaginalis that had spread to a left groin lymph node, the lung and the pleura. He was treated with the immunotherapy drugs ipilimumab and nivolumab. One week after the first cycle, fluid around the right lung had increased and contained mesothelioma cells. After 3 months there was a partial response. It was ongoing at 6 months, with further shrinking of the pleural nodules and fluid. A moderate (Grade 2) rash occurred. Genetic testing found a BAP1 variant that could be inherited or acquired. Among 18 published cases the authors reviewed, one patient had reported asbestos exposure. The authors noted that the benefit of the treatment was unclear because such patients were excluded from the CheckMate 743 trial. They suggested it may be a new treatment option.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of one patient, with a review of 18 previously published cases
• Who was studied: One 80-year-old man with a left scrotal mass and pain, treated in South Australia (author affiliations); plus 18 published cases diagnosed between 1994 and 2022
• Exposure: No known asbestos exposure; 15 pack year smoking history; no scrotal trauma
• Main results:
  • The patient's tumor was diagnosed as malignant epithelioid mesothelioma of the tunica vaginalis with metastases to the left inguinal node, lung and pleura
  • One week after the first cycle of ipilimumab and nivolumab, the right pleural effusion had increased, and fluid testing confirmed malignant cells
  • At 6 months of treatment the patient had an ongoing partial response, with further reduction in pleural nodules and effusion
  • The patient had a moderate (Grade 2) rash, treated with topical corticosteroids and 10 mg oral prednisolone
  • Genomic testing of the patient found a BAP1 frameshift variant of either somatic or germline origin
  • Among the 18 reviewed published cases, one patient had asbestos exposure, one had a smoking history and one had a trauma history
  • Among the 18 reviewed cases, orchiectomy was performed in 83% of patients
  • Among reviewed cases with known prognosis (n = 14), 36% developed recurrence, 80% of those within 3 years
  • Three reviewed patients received systemic therapy, all with cisplatin and pemetrexed
• The authors' conclusion: The authors concluded that ipilimumab and nivolumab after orchiectomy may be a new treatment option for this rare disease, warranting further investigation in a trial setting
• Limitations the authors state:
  • The authors noted there were no efficacy data for this regimen in mesothelioma of the testis or tunica vaginalis, because such patients were excluded from CheckMate 743
  • In the reviewed case reports, the reasons for giving systemic therapy were not documented

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Tunica Vaginalis, Immunotherapy, Ipilimumab (Yervoy), Nivolumab (Opdivo), Orchiectomy, Asbestos Exposure, Ultrasound.

Related studies

• Malignant mesothelioma of the tunica vaginalis: a atypical presentation of a rare scrotal tumour — M.C. Harris, C. Logan, et al. (2019)
• Bilateral Malignant Mesothelioma of Tunica Vaginalis A Case Report on Rare Presentation — A.A. Shaikh, K.V. Naik, et al. (2017)
• Malignant mesothelioma of the tunica vaginalis testis: a rare case and review of literature — M. Drevinskaite, A. Patasius, et al. (2020)
• Malignant mesothelioma of tunica vaginalis testis: Report of a very rare case with review of the literature — E. Trenti, S.M. Palermo, et al. (2018)
• Malignant Mesothelioma of the Tunica Vaginalis: A Rare Neoplasm--Case Report and Literature Review — M. Segura-González, J. Urias-Rocha, et al. (2015)
• Mesothelioma of the tunica vaginalis testis: a rare malignancy mimicking more common inguino-scrotal masses — P.J. Schure, K.C. van Dalen, et al. (2006)

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