SESSION TITLE: Malignant Disease Global Case Reports
SESSION TYPE: Global Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Teratocarcinoma is a human germ cell tumor and indicates the combination of teratoma and embryonal carcinoma. It is an aggressive tumor with an estimated doubling time of 12 hours. It has been hypothesized that the disease process starts in fetal life with abnormal differentiation of fetal primordial germ cells. It is composed of embryonic carcinoma cells, which represent normal cells forming the inner mass of the blastocyst. These cells retain their embryonic nature and continue to proliferate indefinitely because the adult host does not have the mechanism to control their growth. Due to its rarity, data about teratocarcinoma is mostly from murine experiments. Here, we report a case of teratocarcinoma and, due to its aggressive course, led to the demise of the patient.
CASE PRESENTATION: This is a case of a 21-year-old male presenting with sternal pain, 3-pillow orthopnea and non-productive cough for 1 month. Chest CT scan revealed a 10.0 x 12.0 x 14.0 cm anterior mediastinal mass. He has a strong family history for carcinoma with an aunt having breast cancer, grandmother (father side) dying of a brain tumor, grandmother (mother side) with cervical cancer and uncle with colon cancer. Biopsy of the mass is suggestive of a germ cell tumor with teratomatous component but cannot exclude a malignant component. Though beta-HCG and AFP were elevated, chemotherapy was contraindicated due to poor functional status. Patient underwent emergency mediastinostomy and excision of the mass due to severe dyspnea. Unfortunately, patient went to cardiopulmonary arrest intraoperatively and was unable to be resuscitated. Histopathology of the mass had features compatible with mixed embryonal carcinoma and teratocarcinoma with prominent angiosarcomatous elements, which include hair follicle, nerve, cartilage, intestinal and pancreatic components.
DISCUSSION: Germ cell tumors are neoplasms derived from germ cells, which can be cancerous or non-cancerous. Teratocarcinoma accounts for 3-10% of tumors originating in the mediastinum and only 1-5% of all germ cell neoplasms. It is associated with high levels of beta-HCG and alpha-fetoprotein. Patients with an anterior mediastinal mass and a strong family history of carcinoma, a more aggressive approach, maybe more than that of the carcinoma’s behavior is warranted to get ahead of the disease. Though a tissue diagnosis is still warranted to make a diagnosis of a carcinoma, a probable diagnosis of a teratocarcinoma based on the clinical presentation and elevated beta-HCG and AFP may require starting of treatment, considering the short doubling time of the tumor. Radiotherapy may be started particularly in patients presenting with symptoms due to compression of mediastinal structures. Teratocarcinoma is responsive to radiotherapy and chemotherapy while surgery is reserved for small tumors. However, in the late stage of the disease, surgical debulking maybe warranted prior to radiotherapy or chemotherapy. During surgery, there is a high risk of cardiac tamponade when the patient is sedated and placed on the supine position. The large mass will compress the vital mediastinal structures, particularly the great vessels and the heart. Thus, there is a dilemma of doing neoadjuvant chemotherapy prior to surgery or primary surgical debulking/excision followed by chemotherapy. Reports are conflicting, with one doing surgery followed by 13 cycles of chemotherapy versus one doing 3 courses neoadjuvant chemotherapy prior to complete resection of the mass, both being successful. Despite these case reports, much is to be explored regarding teratocarcinoma in humans rather than in laboratory mice. In particular, the urgency of treatment due to the aggressiveness of the disease, may ultimately decide the patient’s outcome.
CONCLUSIONS: “Infestus morbus” or aggressive disease, truly described the nature of teratocarcinoma. The aggressiveness of the disease should be equaled with the same urgency in the diagnosis and treatment of the disease. A high index of suspicion based on clinical data may guide the clinician in starting treatment and may mean life or death of our patient.
Reference #1: Buli-Jaku, F. et al. Of Mice and Men: Teratomas and Teratocarcinomas. Coll Anthropol 2006;30(4):921-4.
Reference #2: Damjanov, I. Teratocarcinoma: neoplastic lessons about normal embryogenesis. Int J Dev Biol 1993;37:39-46.
Reference #3: Hasgekar, N. et al. A neural precursol cell line derived from murine teratocarcinoma. Int J Dev Biol 1996;40:591-7.
DISCLOSURE: The following authors have nothing to disclose: George Paul Habacon, Teresita DeGuia
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