Testicular Mixed Germ Cell Tumor in a Newborn Child: A Rare Case.

Testicular tumors are rare in children but highly treatable and usually curable. Seminomas are extremely rare in prepubertal children. Among nonseminomatous germ cell tumors, the most common are teratomas and yolk sac tumors. Mixed germ cell tumor (MGCT) contains more than one germ cell component. MGCT is very rare in prepubertal age group. Here, we are presenting a case of MGCT in a newborn child with a review of literature. It is the first reported case in the world literature.

INTRODUCTION

Testicular tumors are far more common in adults than in children. For this reason, the management of pediatric testicular tumors has been based on experience in adults. Indeed, testicular tumors in adults and children are similar in many ways. There is a bimodal age distribution for the incidence of testicular tumors, with one peak occurring during the first 2 years of life and a second, larger peak occurring in young adulthood. The incidence of pediatric testis tumors is 0.5–2.0/100,000 children, accounting for 1%–2% of all pediatric tumors.[1] Mixed germ cell tumors (MGCTs) contain more than one germ cell component and various combinations have been reported. Statistical analysis of possible combinations by Mosharafa et al. revealed 10 possible pair combinations with the strongest correlation between teratoma and yolk sac tumor.[2] The average age of presentation for patients with MGCTs is 30 years. Here, we are presenting a case of MGCT in a newborn child and had yolk sac and immature teratoma component.

CASE REPORT

Newborn child presented to us with the complaint of painless right testicular swelling since birth. On examination, it was 7 cm × 6 cm, nontender, irregular in shape, and hard in consistency. There was no family history of cancer. Antenal ultrasounds were normal. Routine blood investigations were within normal limits, including complete blood counts, renal function test, and liver function test. Color Doppler ultrasound examination revealed a 53 mm × 36 mm solid cystic lesion with multiple calcifications, 16 mm × 8 mm testis-like structure that was not well separated from mass with 54 mm × 33 mm hydrocele in the right side. Left testis was 18 mm × 8 mm in the left scrotal sac. There was a 40 mm × 20 mm mixed echogenic lesion in the left adrenal? Hemorrhage. The clinical diagnosis made was enlarged right testicle, possibly neoplastic. Contrast-enhanced computed tomography showed the right enlarged testis with heterogeneous lesion of 44 mm × 38 mm × 42 mm with multiple necrotic foci. It was in favor of diagnosis of germ cell tumor. Left testis was normal with moderate right hydrocele [Figure 1a]. The right suprarenal region showed well-defined cystic hypodense lesion? Nature. There was no lymphadenopathy. In addition, liver, spleen, gallbladder, pancreas, kidneys, major vessels, and urinary bladder were found to be normal. No detectable masses or lymph nodes were seen in the abdomen. Both lung fields and costo-phrenic angles were clear on chest X-ray. Karyotyping was XY.

Figure 1

(a) Contrast-enhanced computed tomography image, (b) intra-operative photo

The values of tumor markers alpha-fetoprotein (AFP) (38,000 ng/ml) and beta-human chorionic gonadotropin (HCG) (15 IU/L) were normal. The patient underwent unilateral orchidectomy through inguinal approach. The right testis was removed together with all its coverings with maximum cord length up to the deep inguinal ring [Figure 1b]. No fixity to the skin was seen. The patient was discharged after 3 days and stitches removed after 8 days. The postoperative period was uneventful. Histopathology report showed MGCT with immature teratoma and yolk sac component. The tumor was extending into the epididymis and involving the tunica albuginea. It was Stage I tumor; hence, we keep the patient under follow-up without chemotherapy.

DISCUSSION

Testicular tumors are a heterogeneous group of neoplasms exhibiting diverse histopathology and can be classified into seminomatous and nonseminomatous germ cell tumor types with variable course and prognosis.[3] Seminomas are extremely rare in prepubertal children. Among nonseminomatous germ cell tumors, the most common are teratomas and yolk sac tumors which account for about 62% and 26% of testicular tumor in infants and toddlers, respectively.[4]

In 1984, Weissbach et al.[5] reviewed the proportion of histologic types prevalent among adult and pediatric testicular tumors. The investigators studied 1062 adult tumors in the Bonn registry and 1169 pediatric tumors extracted from a review of the literature. Among adults, seminomas and MGCTs accounted for 89% of cases, with stromal tumors accounting for 8% and yolk sac tumors and teratomas accounting for 1% each. In contrast, 49% of pediatric tumors were yolk sac tumors, 29% were stromal, 13% were teratomas, and only 9% were seminomas or MGCT. The study included adolescents among the cases of “pediatric tumors.” However, when patients are divided along the line of puberty, virtually no prepubertal tumors are seminomas or MGCT.

Different risk factors have been described for testicular cancer in the early age group. They include prenatal exposure to high estrogen levels during intrauterine life, gonadal dysgenesis, exposure of mother to chemical carcinogens and smoking, trauma, testicular atrophy due to torsion, and orchitis. Family history of cancer is an important risk factor in the early age groups. There was no family history in our case and karyotyping was XY.

Three clinical stages for the determination of extension of the tumor have been described.[6] Stage I is where the tumor is confined to the testis. Invasion of epididymis tunica albuginea, spermatic cord, or scrotum does not change tumor stage but increases the risk of nodal involvement and the risk of recurrence.

In Stage II, the tumor has retroperitoneal lymph node metastasis. Stage III is characterized by supraclavicular lymph nodes, visceral involvement, or persistently elevated tumor marker values. In our case, there was Stage I, so keep in follow-up without chemotherapy.

The majority of patients with testicular tumors present with a painless, hard testicular mass, noted by the patient, a parent, or a healthcare provider. When the physical examination is equivocal, ultrasound is an excellent tool. On occasion, a patient will present with a hydrocele. Since hydroceles are common in children and tumors are rare. However, if a child presents with a hydrocele and the testis cannot be easily palpated, ultrasound should be performed.[7]

Tumor markers typically used in the evaluation and management of testicular tumors include HCG and AFP. Although HCG is elaborated in a significant number of MGCT, this tumor type is vanishingly rare in prepubertal patients. On the other hand, AFP levels are elevated in 90% of patients with yolk sac tumors and can be helpful in the preoperative distinction between yolk sac and other tumors. Since many, if not most, prepubertal tumors are benign, the metastatic evaluation may be deferred until a histologic diagnosis of the primary tumor is obtained. The metastatic evaluation includes chest X-ray or computerized tomography (CT) scan, as well as an abdominal CT scan should be obtained, and postoperative AFP levels should be followed. The serum markers levels were within normal limit in our case.

One of the major paradigm shifts in the management of prepubertal testicular tumors involves the management of the primary tumor. Except for tumors in children older than 6 months with elevated AFP levels (who most likely harbor yolk sac tumors), the initial surgical management of a prepubertal testicular tumor is excisional biopsy with frozen section analysis. This strategy is supported by the fact that, compared with adult tumors, for which inguinal orchiectomy is standard surgical management, a high percentage of prepubertal tumors are benign. In addition, the desire to preserve testicular tissue may be more compelling in a child who has yet to experience puberty. Another change in the management of prepubertal testicular tumors has been a shift away from retroperitoneal lymph node dissection, which was once a standard component of treatment.[8] We do not have frozen section facility at our institute, and hence, we did high inguinal orchidectomy.

Certainly, many of these patients present late, usually with some or the other complications which are difficult to treat and carry bad prognosis. Still, if they can complete the platinum-based chemotherapy, they have a reasonable survival period depending on the complications they have.

Since 1980, 338 testicular tumors have been registered in the Prepubertal Testicular Tumor Registry of the American Academy of Pediatrics, Section on Urology. Of these tumors, 22 occurred in neonates <1 month old, of which 7 (31%) were diagnosed at birth. The distribution of lesions in this group revealed six yolk sac tumors, six gonadal stromal tumors, six juvenile granulosa cell tumors, two gonadoblastomas, one teratoma, and one hamartoma. Although neonatal testis tumors are rare, they should be considered in the differential diagnosis and management of a newborn with a scrotal mass.[9]

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Conflicts of interest

There are no conflicts of interest.