|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 470-472
Mixed germ cell tumor in a teenager: A rare entity
CR Choudhury1, N Bhattacharya2, TD Bhutia1, M Mondal2
1 Department of Surgery, Medical College Hospital, 88 College Street, Kolkata, West Bengal, India
2 Department of Pathology, Medical College Hospital, 88 College Street, Kolkata, West Bengal, India
|Date of Web Publication||18-Feb-2016|
C R Choudhury
Department of Surgery, Medical College Hospital, 88 College Street, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Choudhury C R, Bhattacharya N, Bhutia T D, Mondal M. Mixed germ cell tumor in a teenager: A rare entity. Indian J Cancer 2015;52:470-2
Mixed germ cell tumors (GCTs) contain more than one germ cell component and are much more common than any of the pure histological forms, representing 32-60% of all germ cell tumors. Essentially, any admixture of germ cell tumors, as seen in the pure form, may be seen; one of the most common admixtures being embryonal carcinoma and teratoma. Their average age of presentation is 30 years and they rarely occur in prepubertal patients. Here we report a case of a disseminated mixed germ cell testicular tumor in a 14-year-old prepubertal boy [Figure 1].
The patient presented with pain in the right lumbar region for three months, anorexia, significant weight loss, night sweats, and generalized weakness. He had generalized lymphadenopathy, hepatomegaly, and a lump (5 × 3 cm) in the right lumbar region. The right testis was apparently normal in size, but it was tender. Blood examination showed hemoglobin to be 7 g/dl and serum LDH was 2454 U/L.
An ultrasound showed mild hepatomegaly with multiple hyperechoic lesions in both lobes of the liver. The right kidney was enlarged, with a few ill-defined nodular lesions. Inferior vena cava (IVC) thrombosis and multiple retroperitoneal lymphadenopathy were noted. Ultrasound of the scrotum revealed a heterogeneously hypoechoic (2.1 × 1.7 cm) space-occupying lesion (SOL) in the right testis.
Multiple foci of calcifications were noted in both the testes. Contrast-enhanced computed tomography (CECT) of the whole abdomen confirmed the ultrasound findings, and sections through the thorax showed mediastinal lymphadenopathy and pulmonary nodules [Figure 2] and [Figure 3]. A CT-scan-guided Trucut biopsy of the kidney and liver revealed a metastatic germ cell tumor [Figure 4] and [Figure 5]. A high inguinal right orchidectomy was performed [Figure 6], but unfortunately the patient expired on the first postoperative day, without receiving any further treatment. Histopathological examination of the testis showed a yolk sac tumor with focal areas of embryonal carcinoma and involvement of the spermatic cord [Figure 7]. Immunohistochemistry (IHC) was focally positive for alpha fetoprotein and negative for b-hcg [Figure 8].
|Figure 2: CECT scan of the chest, showing pulmonary nodules and mediastinal lymphadenopathy|
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|Figure 3: CECT scan of the abdomen showing enlarged kidney with hypoechoic lesions|
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|Figure 4: Fine needle aspiration from liver SOL showing clusters of large malignant cells, with large vesicular nuclei, coarse chromatin, and prominent nucleoli, with cytoplasmic vacuolation, ×400|
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|Figure 5: Truecut biopsy of the kidney showing the histology of a metastatic mixed germ cell tumor, H and E, x100|
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Testicular cancer is comprised of approximately 2% of all malignant cancer in men and 10% of all malignant male genitourinary cancers. Jacobsen et al. have shown that mixed germ cell tumors constitute 69% of all non-seminomatous germ cell tumors and 32% of all testicular germ cell tumors. Embryonal carcinoma is the most common component and is often combined with teratoma, seminoma, or yolk sac tumor. The presence of embryonal carcinoma has been associated with a tendency to metastasize. It is well-documented that the presence of yolk sac elements is highly associated with precocious metastases.
Tumor calcifications occurred in 40% of the patients. In our patient, microcalcification was found in both the testes. The incidence of germ cell tumor (GCT) with microlithiasis was reported to be 6-46%. Jun Akatsuka, et al. also reported that the fourteenth case in Japan, was of a 31-year-old male with embryonal cell tumor, with IVC thrombus, involvement of the spermatic cord, and multiple lung nodules. This patient also had similar findings along with metastasis to the liver, kidney, and mediastinum, without any respiratory complaints or lower limb edema.
Germ cell tumors might be primary, metastatic, or multifocal — first reported by Reznik, et al., in 1994. In disseminated cases, a meticulous history, thorough physical examination, and imaging might help to conclude the primary site. However, in our patient, with such a small testicular SOL and mediastinum involvement, it was difficult to ascertain whether the tumor was multifocal or had a primary site that metastasized.
Modern chemotherapy combined with surgery, can achieve a complete cure in over 90% of all patients with malignant testicular germ cell tumors. Even tumors that have already metastasized at the time of orchidectomy are curable by platinum-based multidrug treatment. Studies suggest that the promoter hypermethylation of the RASSF1A and HIC1 genes plays a role in the resistance of GCT, while the transcriptional inactivation of O (6)-methylguanine-DNA methyltransferase (MGMT) by epigenetic alterations confers an exquisite sensitivity to cisplatin.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]