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A 40-year-old man presented with a large left neck mass in the setting of recently treated mixed nonseminomatous germ cell tumor (NSGCT) of the right testicle with metastasis to the retroperitoneal and mediastinal lymph nodes. He was treated with a right radical orchiectomy and 4 cycles of etoposide, ifosfamide, and cisplatin (VIP) chemotherapy with resulting reduction in metastatic tumor burden and normalization of α-fetoprotein and β-human chorionic gonadotropin. The patient’s neck swelling, which initially decreased in size while undergoing chemotherapy, progressively enlarged shortly after concluding treatment. He underwent magnetic resonance imaging and computed tomography of the neck that showed a clearly defined, bulky supraclavicular mass confined to left neck levels 3, 4, and 5 (Figure).
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C. Growing teratoma syndrome
Despite an excellent retroperitoneal and mediastinal tumor response to 4 cycles of VIP chemotherapy, this patient’s left supraclavicular mass continued to enlarge. While chemorefractory metastasis was in the differential diagnosis, normalization of tumor markers and radiographic appearance on magnetic resonance imaging indicated the diagnosis of growing teratoma syndrome (GTS). Surgical resection was performed given the high risk of mechanical compression of surrounding neck vasculature owing to unchecked teratoma growth. Final pathology results demonstrated mature teratoma tissue, confirming the diagnosis of GTS.
Growing teratoma syndrome was first described in 1982 by Logothetis et al,1 in which 6 patients with metastatic germ cell tumors were described to have enlarging abdominal and pulmonary masses despite successful chemotherapy regimens. The criteria by Logothetis et al1 for diagnosing GTS included (1) a normalization of β-human chorionic gonadotropin and α-fetoprotein values, (2) presence of a mass on examination that exhibited continual growth despite chemotherapy treatments, and (3) absence of any NSGCT malignant histologic characteristics on pathological examination.
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Jeremiah C. Tracy, MD, Department of Otolaryngology–Head and Neck Surgery, Tufts Medical Center, 800 Washington St, Box 850, Boston, MA 02111 (firstname.lastname@example.org).
Published Online: September 23, 2021. doi:10.1001/jamaoto.2021.2420
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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