Annals of Oncology Advance Access originally published online on May 25, 2006
Annals of Oncology 2006 17(10):1602-1604; doi:10.1093/annonc/mdl091
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© 2006 European Society for Medical Oncology
letters to the editor |
Mediastinal mature teratoma with an immature component—what about the treatment?
Oncology Institute of Southern Switzerland, Hospital San Giovanni, Department of Oncology, Bellinzona, Switzerland
*(E-mail: egallerani{at}iosi.ch)
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Mediastinal germ cell tumors are broadly classified as benign or malignant. Benign tumors include mature teratomas and mature teratomas with an immature component of <50%. Usually, mediastinal teratomas are the most frequent mediastinal germ cell tumors, while immature teratomas are very rare [1].
The mediastinal teratomas can be seen in any age group but most commonly occur in adults from 20 to 40 years of age [2]. Histologically, teratomas may be solid or cystic in appearance and are often referred to as dermoid cysts if unilocular. The majority of mediastinal teratomas is composed of mature ectodermal, mesodermal, and endodermal elements and exhibits a benign course [3]. Immature teratomas phenotypically may appear as a malignancy derived from all the embryological elements. These latter tumors behave aggressively and generally are not responsive to systemic therapy and their prognosis is influenced by the anatomic site of the tumor, patient age, and the fraction of the tumor that is immature. In patients younger than 15 years, immature teratomas behave similarly to their mature counterparts [4]. In older patients, they may behave as highly malignant tumors [5].
Determination of serum tumor markers (
-fetoprotein AFP and human beta-choriogonadotropin HCG) is important in the diagnosis and follow-up of mediastinal germ cell tumors. Differently from immature teratomas, patients with benign teratomas have normal markers. Long-term follow up of AFP and HCG levels, clinical controls and controls by imaging methods are indicated in children with an immature or malignant component of the tumor [6].
For mature and differentiated teratomas the radical surgical removal is the only curative treatment, because unnecessary delay may result in the rupture of tumors or in malignant degeneration. The prognosis after surgical removal of the tumor is good and there is no recurrence of tumor after complete excision [7]. For immature teratomas the main options are primary resection and chemotherapy [8, 9].
In the literature patients older than 15 years with immature mediastinal teratomas were classified into survivor and non-survivor groups, and the characteristics in each group were evaluated [10]. In the survivor group, the tumor was completely resected or patients showing a high level of alpha-fetoprotein were treated by pre-operative chemotherapy, while the negative marker patients were treated by post-operative chemotherapy. In the non survivor group, the tumor was resected incompletely, or no chemotherapy was performed. In patients more than 15 years old with immature mediastinal teratomas, complete surgical resection of the tumor is indispensable but the association with the chemotherapy prolongs survival.
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case report |
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We report a case of a massive mediastinal teratoma in an 18 year-old man who presented with a short history of thoracic precordial pain and inflammatory syndrome accompanied by fever and asthenia. The x-ray documented an abnormal shadow in the mediastinum. A CT-scan revealed a bulky mass of 7 x 9 cm in the superior mediastinal region, extended into the left pleural cavity, adherent to the aorta and compressing the pulmonary artery (Figure 1).
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The serum alpha fetoprotein (AFP) was elevated to 131 ng/ml (n.v. <8.6 ng/ml), ß-HCG and LDH were normal. A bilateral ultrasound of the testis was normal, resulting in the need of a fine-needle biopsy of the mass that at the beginning was not diagnostic. As a consequence the mediastinal biopsy in mediastinoscopy was performed revealing tissues compatible with mature teratoma, despite the elevation of AFP.
The tumor was treated by surgical resection, with a final surprising pathological diagnosis of a mediastinal mature teratoma (80%) and immature with a neuroepithelial component (20%).
The surgeon could not warrant the radicalness of the surgical intervention, while the pathologist could not evaluate the tumor margins. The tumor was widely sampled and it was composed of cystic alternating solid components with mature tissue from different histogenesis (skin, sweat glands, gastrointestinal, pancreatic, osteocartilagineous tissue and neuroepithelial part in the immature element). Immuno-histochemistry was positive for PLAP, -FP, ß-HCG, pancyokeratin, chromogranin, snaptophysin, GFAP, neuro-filaments, CD99, MIB-1.
After the excision, the elevated serum alpha fetoprotein (AFP) levels dropped to 42.8 ng/ml after 1 week from surgery up to a normal value.
The PET-CT scan a month after surgery showed a mediastinal activity (DD residual-tumor or inflammation tissue) (Figure 2).
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Because of the association between the immature component, the extragonadic position, the high level of serum AFP and the lack of a sure radicalness, this teratoma was suspected to be a malignant tumor, and the patient underwent chemotherapy. He was treated with three cycles of BEP as adjuvant chemotherapy and after two cycles another PET-CT scan was performed documenting no residual glucose-uptake and still normal AFP value. The young man responded favourably and was well after a month's follow-up.
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discussion |
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In consideration of the pathology, no frankly malignant elements were found in this case. The judgment can be difficult to distinguish immature from malignant mesodermal components. However, despite the pathology, the presence of raised AFP pre-operatively suggests there was a malignant component present originally. Taken with the bulk of the tumor and the uncertainty of resection, this case can be considered a good case for adjuvant chemotherapy. The diagnostic difficulties of the mediastinal approach that at the beginning revealed only mature teratoma prevented a neoadjuvant treatment.
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conclusions |
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Mediastinal immature teratoma has a worse prognosis in comparison with the situation of metastatic retroperitoneal lymph node primary testicular germ cell cancer treated with two adjuvant cycles of chemotherapy. Given the greater drug resistance of mediastinal primaries the question is whether to recommend more than two cycles of BEP as adjuvant.
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References |
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1. Dulmet EM, Macchiarini P, Suc B, et al. (1993) Germ cell tumors of the mediastinum: a 30 year experience. Cancer 72:1994.
2. Arai K, Ohta S, Suzuki M, et al. (1997) Primary immature mediastinal teratoma in adulthood. Eur J Surg Oncol 23:164–67.[CrossRef][ISI][Medline]
3. Moeller KH, Rosado-de-Christenson ML, Templeton PA. (1997) Mediastinal mature teratoma: imaging features. AJR Am J Roentgenol 169:4985–990.
4. Rygl M, Snajdauf J, Zeman L, et al. (2001) Mediastinal teratomas in children. Rozhl Chir 80:12624–627.[Medline]
5. Hiroshima K, Toyozaki T, Iyoda A, et al. (2001) Apoptosis and proliferative activity in mature and immature teratomas of the mediastinum. Cancer 92:71798–806.[CrossRef][ISI][Medline]
6. Taniyama K, Ohta S, Suzuki H, Matsumoto M, et al. (1992) Alpha-fetoprotein-producing immature mediastinal teratoma showing rapid and massive recurrent growth in an adult. Acta Pathol Jpn 42:12911.[Medline]
7. Smahi M, Achir A, Chafik A, et al. (2000) Mature teratoma of the mediastinum. Ann Chir 125:10965–971.[CrossRef][ISI][Medline]
8. Iyoda A, Hiroshima K, Yusa T, et al. (2000) The primary mediastinal growing teratoma syndrome. Anticancer Res. 20:5C3723–3726.[ISI][Medline]
9. McLeod NP, Vallely MP, Mathur MN. (2005) Massive immature mediastinal teratoma extending into the left pleural cavity. Heart Lung Circ 14:145–47.
10. Lakhoo K, Boyle M, Drake DP. (1993) Mediastinal teratomas: review of 15 pediatric cases. J Pediatr Surg 28:91161–1164.[CrossRef][ISI][Medline]
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