Embryonal Carcinoma


Embryonal carcinoma (EC) is a relatively rare type of nonseminomatous germ cell tumor that usually occurs in the ovaries and testes. EC is a malignant tumor that can be aggressive and spread to other parts of the body.

In males, the main presenting symptom of EC is a palpable testicular mass or asymmetric testicular enlargement. The tumor can also present with signs and symptoms associated with the presence of metastatic disease such as low back pain, dyspnea, cough, hemoptysis, hematemesis, and neurologic symptoms. The male counterpart of EC has a normal level of alpha fetoprotein (AFP). If elevated, the tumor suggests more of a mixed germ cell tumor, with the elevation of alpha fetoprotein produced by the yolk sac component. The average age at diagnosis is 31 years and typically presents with a painful testicular mass. Almost two-thirds of cases have metastasis at diagnosis. Testicular EC occurs mostly (84%) as a component of a mixed germ cell tumor, with only 16% being pure.

In females, EC is quite rare, accounting for 3% of all ovarian germ cell tumors. The median age at diagnosis is fifteen years. Symptoms and signs might vary and may include sexual precocity and abnormal uterine bleeding. Alpha fetoprotein and human chorionic gonadotropin (HCG) might be elevated. Children develop large unilateral tumors with a median diameter of 17 centimeters. Metastasis is present in 40% of cases upon diagnosis. Clinically, females present with abdominal pain, abdominal distension, or a pelvic mass.
In both males and females, imaging with US, CT-Scan, and MRI is needed, including a metastatic workup of chest and CNS.

EC can also arise in extragonadal sites such as the retroperitoneum, mediastinum, central nervous system, liver, and gastrointestinal tract. Primary intracranial embryonal carcinoma is a rare brain tumor. EC accounts for 5% of all intracranial tumors. Eighty percent of these tumors tend to occur along the midline, such as the pineal, suprasellar region, hypothalamus, and third ventricle. Preoperative imaging examination, blood serum, and cerebrospinal fluid levels of AFP and HCG can support the diagnosis. Subtotal to total removal with good preservation of important structures can be achieved in this tumor resection. EC has a poor prognosis in the CNS.

Pathologically, EC is a poorly defined tumor with associated hemorrhage and necrosis. Microscopic features include indistinct cell borders, variable architecture, balls of cells surrounded by empty space on three sides, nuclear overlap, and necrosis. Solid (55%), glandular (17%), and papillary (11%) are the most common primary patterns.

Initial management of EC is surgical excision, meaning radical orchiectomy through an inguinal approach in males and unilateral adnexectomy in females. In stage 1 disease, it is the curative treatment in 75% of all cases. The patient should be counseled for a possible biopsy of the contralateral adnexa when appropriate.

Non-seminomatous germ cell tumors are the most sensitive testicular and ovarian cancers to cisplatin-based chemotherapy. From three to four to six cycles of chemotherapy with BEP (bleomycin, etoposide, and cisplatin) is regarded as the gold-standard regimen for the treatment of germ cell tumors at all stages of disease. After completion of chemotherapy, tumor markers are repeated to see the decline in values, along with a contrast-enhanced CT scan to evaluate for any residual mass. Retroperitoneal lymph node dissection with adjuvant chemotherapy is the mainstay of managing low-stage non-seminomatous germ cell tumors in the United States.

Overall, fertility is reduced by an average of 30% after chemotherapy treatment. Fertility preservation in females is of utmost importance, including cryopreservation of oocytes, ovarian tissue, or embryos. A question in issue is how long after chemotherapy could women conceive. Six months is the time needed for human oocyte maturation from a dormant state to fully mature. Cancer patients are advised not to conceive until six months from the completion of chemotherapy. Many studies suggest that the right time is 24 months after the end of the last cycle of chemotherapy.

References:
1- Kelly GM, Gatie MI. Mechanisms Regulating Stemness and Differentiation in Embryonal Carcinoma Cells. Stem Cells Int. 2017:3684178. doi: 10.1155/2017/3684178, 2017
2- Jiang T, Raynald, Yang H, Wang J, Du J, Zhang W, Shao Q, Li C. Primary intracranial embryonal carcinoma in children: report of two cases with review of the literature. Int J Clin Exp Pathol. 10(11):10700-10710, 2017
3- Cerovac A, Ljuca D, Nevacinovic E, Tulumovic A, Iljazovic E. Giving Birth After Fertility Sparing Treatment of Embrional Carcinoma Figo III C: Case Report and Literature Review. Med Arch. 72(5):371-373, 20185
4- Seema MaheshÊ 1 ,ÊMahesh Mallappa,ÊGeorge Vithoulkas: Embryonal Carcinoma with Immature Teratoma: A Homeopathic Case Report. Complement Med Res. 25(2):117-121, 2018
5- Kattuoa ML, Dunton CJ. Yolk Sac Tumors. 2023 Feb 4. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024


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