Ann Thorac Surg 2006;82:1906-1908
© 2006 The Society of Thoracic Surgeons
Case Reports
Treatment of Mediastinal Immature Teratoma in a Child With Precocious Puberty and Klinefelter's Syndrome
Chen-Kun Chen, MDa,
Yih-Leong Chang, MDb,
Shiann-Tarng Jou, MDc,
Yu-Ting Tseng, MDa,
Yung-Chie Lee, MD, PhDd,*
a Department of Surgery, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
b Department of Pathology, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
c Department of Pediatry, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
d Department of Surgery and Traumatology, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
Accepted for publication March 27, 2006.
* Address correspondence to Dr Lee, Department of Surgery, No. 7, Jhongshan S Rd, Jhongjheng District, Taipei City, R.O.C. 100 Taiwan. (Email: wuj{at}ha.mc.ntu.edu.tw).
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Abstract
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Teratoma is the most common germ cell tumor, which can be divided into the mature and the immature histologically. Concurrent Klinefelter's syndrome may be overlooked in a patient with a germ cell tumor. This is because the tumor that secrets alpha-fetoprotein and beta human chorionic gonadotropin can mimic puberty in a patient with Klinefelter's syndrome, masking the usual clinical signs. In reviewing the literature on the subject, the role of neoadjuvant and adjuvant chemotherapy remains ill-defined for the immature teratoma. Age-dependent prognosis seems to demonstrate that children with immature teratomas have a better outcome. We share the experience of treating a child with immature teratoma with surgical excision alone, and it ended in a local recurrence.
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Introduction
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The association of mediastinal germ cell tumors (GCTs) and Klinefelter's syndrome (KS) is well established. The hypogonadal hypogonadism in patients with KS can be masked by precocious puberty resulting from chorionic gonadotropin secreted by GCTs. Histologically, mediastinal GCTs are comprised of three distinct groups of neoplasm: (1) teratomas, (2) seminomas, and (3) nonseminomatous germ cell tumors. Teratomas could be further divided into the mature and the immature. Among these, immature teratomas account for only 1% of all mediastinal teratomas [1].
In reviewing the literature on the subject, the role of neoadjuvant and adjuvant chemotherapy remains ill-defined for the immature teratoma. We shared the experience of treating a child with immature teratoma with surgical excision alone, and it ended in a local recurrence.
A 5-year-old boy showed symptoms of precocious puberty, including enlargement of the penis, development of pubic hair, facial comedoes, and deepening of the voice. The bone age was measured between the age of 11 years, 6 months and the age of 12 years, 6 months. The alpha-fetoprotein (AFP) was up to 331.72 ng/mL (normal, 20 ng/mL) and beta human chorionic gonadotropin (ß-hCG) to 19.6 mIu/mL (normal, 5 mIu/mL). The gonadotropin releasing hormone simulating test evoked no appreciable rise in follicle stimulating hormone or luteinizing hormone. The chest computer tomography demonstrated an anterior mediastinal 7 x 5 x 5 cm tumor (Fig 1A). The biopsy showed myxoid tissue and cytokeratin positive cystic lining epithelium. No evidence of malignant or primitive tissue was found. Tests were checked and no tumor was found.
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Fig 1. The chest computer tomography demonstrated an anterior mediastinal tumor in a 5-year-old child of precocious puberty and Klinefelter's syndrome (A). The cut surface of the resected specimen showed a feature suggestive of mature teratoma (B).
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A chromosome study showed 47 XXY karyotype. The diagnosis of KS was established. The tumor (Fig 1B) was completely resected through a median sternotomy and the pathology revealed a mostly mature teratoma with a small portion of primitive neuro-epithelium, which was immunoreactive to neuron-specific enolase, synaptophysin, AFP, and ß-hCG. Other portions of the teratomatous tumor were all mature (Fig 2). The AFP and ß-hCG were gradually down to normal range after 7 months postoperatively.
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Fig 2. The pathology showed a small focus of primitive neuroectodermal tissue in an overwhelming majority of mature teratomatous components.
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However, recurrent signs of precocious puberty were found after 9 months postoperatively. The AFP increased to 23.36 ng/mL and the ß-hCG increased to 25.1 mIu/mL. The chest computer tomography revealed an anterior mediastinal 5 x 3 x 3 cm tumor. Preoperative chemotherapy, Bleomycin, Etoposide, and Cisplatin protocol (Bleomycin 15 Iu/m2, Etoposide 100 ng/m2, Cisplatin 100 mg/m2) was arranged. The AFP was down to 4.41 ng/mL and ß-hCG was < 0.5 mIu/mL. Tumor excision was done 3 weeks after chemotherapy, and pathology revealed a totally mature teratoma. Adjuvant chemotherapy with BEP was added. No recurrence was found to date with 6 months of follow-up after the second operation.
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Comment
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Immature teratomas, which are usually considered to be nonhormone-secreting, have been reported with elevated ß-hCG and AFP [1]. Arai and colleagues [2] considered that AFP-producing cells are of high malignancy, based on the fact that the prognosis of teratoma containing AFP-producing cells seems to be poor. The high level of preoperative AFP in our case may therefore imply the potential malignancy of this tumor.
There is no standard treatment for the mediastinal immature teratomas. Radical resection is indicated, but the role of neoadjuvant and adjuvant chemotherapy remains uncertain. Unlike ovarian or testicular immature teratomas, primary mediastinal immature teratomas are age-dependent. Patients who are older than 15 years of age have a poor prognosis, despite adjuvant therapy compared with those who are less than 15 years of age [1, 3]. However, due to recent advances in chemotherapy, long-term survival in adults has been reported. Arai and colleagues [2] reviewed that for patients older than 15 years of age, long-term survival can be expected under complete resection combined with preoperative chemotherapy for those of high level AFP, and with postoperative chemotherapy for those of negative tumor markers. Most authors seem to accept that immature teratomas in children have a similar, benign course to mature teratomas. Neyssa and colleagues [4] reported surgical excision as a safe and effective treatment for 80% to 100% of children who are less than 21 years of age with immature teratoma. Another case report by Lisa and colleagues [5] revealed a successful follow-up in a 4.5-year-old boy of a teratoma with foci of malignant mixed GCT elements, who was managed with surgical excision alone.
In our case, complete surgical excision alone did not result in long-term disease-free survival. The tumor recurred with an elevated hormone level. The second pathology revealed a totally mature teratoma, whereas neoadjuvant chemotherapy worked on reducing the hormone level, despite the general acceptance that mature teratomas were insensitive to chemotherapy. There were a couple of possible assumptions: (1) These hormone-producing cells could be of malignant potential and were responsible for the recurrence and were sensitive to chemotherapeutic agents. (2) There may be ectopic immature tissues, other than the mediastinum that were responsible for the recurrence and sensitivity to the chemotherapeutic agents.
We shared our experience of treating this 5-year-old boy with mediastinal teratoma of a small focal immature component and elevated AFP and ß-hCG. Surgical excision alone may not allow a long-term result and neoadjuvant or adjuvant chemotherapy could be considered. More reports and longer follow-up may be needed for definite conclusion of this clinical uncertainty.
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References
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- Carter D, Bibro MC, Touloukian RJ. Benign clinical behavior of immature mediastinal teratoma in infancy and childhood Cancer 1982;49:398-402.[Medline]
- Arai K, Ohta S, Suzuki M, et al. Primary immature teratoma in adulthood Eur J Surg Oncol 1997;23:64-67.[Medline]
- Dulmet EM, Macchiarini P, Suc B, et al. Germ cell tumors of the mediastinum: a 30-year experience Cancerk 1993;72:1894.
- Neyssa MM, Barbara C, Roger G, et al. Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: a pediatric oncology group/children's cancer group intergroup study J Clin Oncol 1999;17:2137-2143.[Abstract/Free Full Text]
- Lisa B, Conrad VF, Elizabeth C, et al. Mediastinal polyembryoma associated with Klinefelter syndrome J Pediatr Hematol Oncol 2003;25:321-323.[Medline]
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Abstract
Introduction
