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Rapidly Enlarging Neck Mass in a Patient With Testicular Cancer

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Jeremiah C. Tracy, MD, Department of Otolaryngology–Head and Neck Surgery, Tufts Medical Center, 800 Washington St, Box 850, Boston, MA 02111 (jtracy@tuftsmedicalcenter.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.

References
1.
Logothetis  CJ , Samuels  ML , Trindade  A , Johnson  DE .  The growing teratoma syndrome.   Cancer. 1982;50(8):1629-1635. doi:10.1002/1097-0142(19821015)50:8<1629::AID-CNCR2820500828>3.0.CO;2-1PubMedGoogle ScholarCrossref
2.
Lee  DJ , Djaladat  H , Tadros  NN ,  et al.  Growing teratoma syndrome: clinical and radiographic characteristics.   Int J Urol. 2014;21(9):905-908. doi:10.1111/iju.12466PubMedGoogle ScholarCrossref
3.
Nimkin  K , Gupta  P , McCauley  R , Gilchrist  BF , Lessin  MS .  The growing teratoma syndrome.   Pediatr Radiol. 2004;34(3):259-262. doi:10.1007/s00247-003-1045-zPubMedGoogle ScholarCrossref
4.
Nassiri  N , Shah  A , Daneshmand  S .  Growing teratoma syndrome of testicular origin.   Urology. 2019;123:20-23. doi:10.1016/j.urology.2018.05.015PubMedGoogle ScholarCrossref
5.
Hiester  A , Nettersheim  D , Nini  A , Lusch  A , Albers  P .  Management, treatment, and molecular background of the growing teratoma syndrome.   Urol Clin North Am. 2019;46(3):419-427. doi:10.1016/j.ucl.2019.04.008PubMedGoogle ScholarCrossref
6.
Laguna  M , Albers  P , Algaba  F ,  et al. EAU guidelines on testicular cancer. Accessed August 17, 2021. https://uroweb.org/wp-content/uploads/EAU-Guidelines-on-Testicular-Cancer-2020.pdf
7.
Kesler  KA , Patel  JB , Kruter  LE ,  et al.  The “growing teratoma syndrome” in primary mediastinal nonseminomatous germ cell tumors: criteria based on current practice.   J Thorac Cardiovasc Surg. 2012;144(2):438-443. doi:10.1016/j.jtcvs.2012.05.053PubMedGoogle ScholarCrossref
8.
André  F , Fizazi  K , Culine  S ,  et al.  The growing teratoma syndrome: results of therapy and long-term follow-up of 33 patients.   Eur J Cancer. 2000;36(11):1389-1394. doi:10.1016/S0959-8049(00)00137-4PubMedGoogle ScholarCrossref
9.
Motzer  RJ , Amsterdam  A , Prieto  V ,  et al.  Teratoma with malignant transformation: diverse malignant histologies arising in men with germ cell tumors.   J Urol. 1998;159(1):133-138. doi:10.1016/S0022-5347(01)64035-7PubMedGoogle ScholarCrossref
10.
Jones  TD , Wang  M , Sung  MT ,  et al.  Clonal origin of metastatic testicular teratomas.   Clin Cancer Res. 2006;12(18):5377-5383. doi:10.1158/1078-0432.CCR-06-0444PubMedGoogle ScholarCrossref
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