European Journal of Radiology, 12 (1991) 138-140 Elsevier
138
EURRAD
00142
The growing teratoma syndrome: radiologic findings in four cases P. Coscojuela’, J. Llauger, C. PCrez, J. Germh and E. Casttier Departments of ‘Radiodiagnosticsand 20ncology Hospital de la Santa Creu i Sant Pau, Barcelona, Spain (Received
14 August 1990; accepted
Key words: Testis, neoplasm;
after revision 3 November
Testis, CT; Growing teratoma
1990)
syndrome
Abstract Radiologic findings in four patients with metastatic mixed germ-cell tumors of the testis are presented. In all cases metastases had increased in size following chemotherapy in spite of normal biomarker levels, and histological study of the residual masses showed mature teratoma with an absence ofmalignant cells, thus confirming the diagnosis ofgrowing teratoma syndrome. At present CT appears to be the best technique both for diagnosis and follow-up in these patients. In one case, the growing teratoma syndrome presented as cavitation of a pulmonary nodular lesion, a finding rarely described in the literature, and with CT demonstration not reported.
Introduction Diagnostic criteria of the growing teratoma syndrome have recently been established in the literature [ 11. This syndrome occurs in nonseminomatous germcell tumors of the testis following chemotherapy and is characterized by an increasing metastatic lesion with normal biomarker levels. Histological examination reveals mature teratoma without malignant cells. The growing mature teratoma is unresponsive to chemotherapy; however, good results are obtained with surgical excision. Here we present the clinical and radiological findings in four patients with growing teratoma syndrome. Results Four patients with nonseminomatous germ-cell tumors of the testis (age 17-34 years) were studied. Orchiectomy was performed in all cases. Plain chest radiographs, thoracic and abdominal CT as well as determination of biomarker levels (a-fetoprotein and P-HCG) were performed. Metastases were localized Address for reprints: Dr. J. Llauger, Servicio de Radiodiagnostico, Hospital de la Santa Creu i Sant Pau, Av. San Antonio M. Claret, 167, 08025 - Barcelona, Spain. 0720-048X/91/%03.50
0 1991 Elsevier Science Publishers
exclusively in the retroperitoneum in two cases, in the retroperitoneum and lung in one case, and in the lung in the remaining case (Fig. 2). Following chemotherapy, biomarkers returned to normal in all patients. Nevertheless, the metastases, which had a cystic appearance, increased in size. In one case (Fig. l), septa were observed within the mass. In the case with abdominal and pulmonary dissemination, the retroperitoneal mass and some pulmonary nodules increased in size, while others either decreased in size In the patient with pulmonary or disappeared. metastases, all nodules disappeared, except for a left basal, which increased in size and cavitated (Fig. 2). Surgical excision of the lesions was performed in all patients. Histologic examination revealed only mature teratoma with extensive cystic zones. No evidence of malignancy was found. All patients have been free of disease for between 2 and 6 years. Discussion Testicular tumors represent 1% of all neoplasms in the male. However, in the 20-30 year age group they are the most common carcinoma and the fourth cause of death due to neoplastic disease [3]. 90 % of testicular tumors are of germ-cell origin, 40 % being seminomas and 50% nonseminomas. Prognosis
B.V. (Biomedical
Division)
Fig. 1. Retroperitoneally growing teratoma syndrome (a) CT reveals right paracaval retroperitoneal solid mass. (b) Increasing treatment is evident. The mass has a more cystic appearance and causes right hydronephrosis.
size after
Fig. 2. Pulmonary growing teratoma. (a) Admission CT shows multiple solid masses in both pulmonary lower lobes. (b) Postchemotherapy CT shows no lesions in the right lower lobe. The metastatic nodule in the left lower lobe has increased in size and cavitated, showing an air/fluid level.
for these patients has improved greatly since the introduction of chemotherapy [4]. Testicular tumors appear to primarily metastasize to the retroperitoneal lymph nodes. A hematogenous route with pulmonary metastases is less frequent. Nodal metastases from nonseminomatous tumors are characterized by biomarker elevation in 60-90 y0 of cases and by retroperitoneal masses at CT. Chemotherapy usually leads to a return to normal biomarker levels and a reduction or disappearance of metastases at CT. Residual masses are observed in 24-60x of cases [5-71. Reduced masses with normal biomarker levels usually represent mature teratoma and/or fibrotic components without malignant potential. Enlarged metastases with elevated biomarkers indicate tumor persistence and the need for continued treatment. In a small percentage of cases, enlargement of the
metastases is associated with normal biomarkers. If histologic examination of the lesions shows only mature teratoma, they meet the diagnostic criteria of growing teratoma syndrome [l-2]. Well-differentiated cells of the mature teratoma may result from the evolution of the malignant tumor (spontaneously or as a result of chemotherapy) or from the growth of mature cells that already exist in the tumor and which are not affected by treatment. The incidence of growing teratoma syndrome is low (1.9% of nonseminomatous tumors with metastases treated with chemotherapy). The majority of the 14 cases described to date had retroperitoneal localization (9 cases). Four cases involving the lung and one involving the cervical lymph nodes have also been described [ 231. Until now CT has been the most efficient technique
140
for follow-up in patients with testicular neoplasm and therefore for diagnosis of the growing teratoma syndrome, which is characterized by an increase in size and a decrease in the density of the lesion, giving the mass a cystic appearance. However, MRI promises to be the optimal follow-up modality. CT demonstration of enlargement and cavitation of a pulmonary nodule as one form of presentation of the growing teratoma syndrome has not been previously described in the radiological literature. However, Logothetis et al. [ 21 described one case that had an enlarging right pulmonary mass, the cystic nature of which was confirmed by transthoracic needle aspiration and injection of contrast material. Tumor masses presenting with the growing teratoma syndrome do not respond to chemotherapy. Surgical excision is indicated to confirm benignity, to avoid malignant degeneration or dissemination, and also to solve possible mechanical complications, mainly obstructive uropathy or caval compression [9].
References 1 Lorigan JG, Eftekhari F, David CT, Shirkhoda A. The growing teratoma syndrome: an unusual manifestation of treated, nonseminomatous germ cell tumors of the testis. AJR 1988; 151: 325-329. 2 Logothetis CJ, Samuels ML, Trindade A, Johnson DE. The growing teratoma syndrome. Cancer 1982; 50: 1629-1635. 3 Mostoti FK. Testicular tumors, epidemiology etiology and pathologic features. Cancer 1973; 32: 1186. 4 Wittes RE, Yagoda A, Silvay 0 et al.. Chemotherapy of germ cell tumors of the testis. Cancer 1976; 37: 637-645. 5 Glowalla M. Residual masses after successful chemotherapy for testicular carcinoma. AJR 1986; 146: 1184-l 186. 6 Stomper PC, Jochelson MS, Friedman EL et al. Evaluation of advanced seminoma treated with chemotherapy. AJR 1986; 745-748. 7 Stomper PC, Jochelson MS, Garnick MB, Richie JP. Residual abdominal masses after chemotherapy for nonseminomatous testicular cancer: correlation ofCI and histology. AJR 1985; 145: 743-746. 8 Hong WK, Wittes RE, Hajdu ST et al.. The evolution of mature teratoma from malignant testicular tumors. Cancer 1977; 40: 2987-2992. 9 Loehrer PJ, Mandelbaum I, Hui S et al. Resection of thoracic and abdominal teratoma in patients after cisplatin-based chemotherapy for germ cell tumor. J Thorac Cardiovasc Surg 1986; 92: 676-683.
138
EURRAD
00142
The growing teratoma syndrome: radiologic findings in four cases P. Coscojuela’, J. Llauger, C. PCrez, J. Germh and E. Casttier Departments of ‘Radiodiagnosticsand 20ncology Hospital de la Santa Creu i Sant Pau, Barcelona, Spain (Received
14 August 1990; accepted
Key words: Testis, neoplasm;
after revision 3 November
Testis, CT; Growing teratoma
1990)
syndrome
Abstract Radiologic findings in four patients with metastatic mixed germ-cell tumors of the testis are presented. In all cases metastases had increased in size following chemotherapy in spite of normal biomarker levels, and histological study of the residual masses showed mature teratoma with an absence ofmalignant cells, thus confirming the diagnosis ofgrowing teratoma syndrome. At present CT appears to be the best technique both for diagnosis and follow-up in these patients. In one case, the growing teratoma syndrome presented as cavitation of a pulmonary nodular lesion, a finding rarely described in the literature, and with CT demonstration not reported.
Introduction Diagnostic criteria of the growing teratoma syndrome have recently been established in the literature [ 11. This syndrome occurs in nonseminomatous germcell tumors of the testis following chemotherapy and is characterized by an increasing metastatic lesion with normal biomarker levels. Histological examination reveals mature teratoma without malignant cells. The growing mature teratoma is unresponsive to chemotherapy; however, good results are obtained with surgical excision. Here we present the clinical and radiological findings in four patients with growing teratoma syndrome. Results Four patients with nonseminomatous germ-cell tumors of the testis (age 17-34 years) were studied. Orchiectomy was performed in all cases. Plain chest radiographs, thoracic and abdominal CT as well as determination of biomarker levels (a-fetoprotein and P-HCG) were performed. Metastases were localized Address for reprints: Dr. J. Llauger, Servicio de Radiodiagnostico, Hospital de la Santa Creu i Sant Pau, Av. San Antonio M. Claret, 167, 08025 - Barcelona, Spain. 0720-048X/91/%03.50
0 1991 Elsevier Science Publishers
exclusively in the retroperitoneum in two cases, in the retroperitoneum and lung in one case, and in the lung in the remaining case (Fig. 2). Following chemotherapy, biomarkers returned to normal in all patients. Nevertheless, the metastases, which had a cystic appearance, increased in size. In one case (Fig. l), septa were observed within the mass. In the case with abdominal and pulmonary dissemination, the retroperitoneal mass and some pulmonary nodules increased in size, while others either decreased in size In the patient with pulmonary or disappeared. metastases, all nodules disappeared, except for a left basal, which increased in size and cavitated (Fig. 2). Surgical excision of the lesions was performed in all patients. Histologic examination revealed only mature teratoma with extensive cystic zones. No evidence of malignancy was found. All patients have been free of disease for between 2 and 6 years. Discussion Testicular tumors represent 1% of all neoplasms in the male. However, in the 20-30 year age group they are the most common carcinoma and the fourth cause of death due to neoplastic disease [3]. 90 % of testicular tumors are of germ-cell origin, 40 % being seminomas and 50% nonseminomas. Prognosis
B.V. (Biomedical
Division)
Fig. 1. Retroperitoneally growing teratoma syndrome (a) CT reveals right paracaval retroperitoneal solid mass. (b) Increasing treatment is evident. The mass has a more cystic appearance and causes right hydronephrosis.
size after
Fig. 2. Pulmonary growing teratoma. (a) Admission CT shows multiple solid masses in both pulmonary lower lobes. (b) Postchemotherapy CT shows no lesions in the right lower lobe. The metastatic nodule in the left lower lobe has increased in size and cavitated, showing an air/fluid level.
for these patients has improved greatly since the introduction of chemotherapy [4]. Testicular tumors appear to primarily metastasize to the retroperitoneal lymph nodes. A hematogenous route with pulmonary metastases is less frequent. Nodal metastases from nonseminomatous tumors are characterized by biomarker elevation in 60-90 y0 of cases and by retroperitoneal masses at CT. Chemotherapy usually leads to a return to normal biomarker levels and a reduction or disappearance of metastases at CT. Residual masses are observed in 24-60x of cases [5-71. Reduced masses with normal biomarker levels usually represent mature teratoma and/or fibrotic components without malignant potential. Enlarged metastases with elevated biomarkers indicate tumor persistence and the need for continued treatment. In a small percentage of cases, enlargement of the
metastases is associated with normal biomarkers. If histologic examination of the lesions shows only mature teratoma, they meet the diagnostic criteria of growing teratoma syndrome [l-2]. Well-differentiated cells of the mature teratoma may result from the evolution of the malignant tumor (spontaneously or as a result of chemotherapy) or from the growth of mature cells that already exist in the tumor and which are not affected by treatment. The incidence of growing teratoma syndrome is low (1.9% of nonseminomatous tumors with metastases treated with chemotherapy). The majority of the 14 cases described to date had retroperitoneal localization (9 cases). Four cases involving the lung and one involving the cervical lymph nodes have also been described [ 231. Until now CT has been the most efficient technique
140
for follow-up in patients with testicular neoplasm and therefore for diagnosis of the growing teratoma syndrome, which is characterized by an increase in size and a decrease in the density of the lesion, giving the mass a cystic appearance. However, MRI promises to be the optimal follow-up modality. CT demonstration of enlargement and cavitation of a pulmonary nodule as one form of presentation of the growing teratoma syndrome has not been previously described in the radiological literature. However, Logothetis et al. [ 21 described one case that had an enlarging right pulmonary mass, the cystic nature of which was confirmed by transthoracic needle aspiration and injection of contrast material. Tumor masses presenting with the growing teratoma syndrome do not respond to chemotherapy. Surgical excision is indicated to confirm benignity, to avoid malignant degeneration or dissemination, and also to solve possible mechanical complications, mainly obstructive uropathy or caval compression [9].
References 1 Lorigan JG, Eftekhari F, David CT, Shirkhoda A. The growing teratoma syndrome: an unusual manifestation of treated, nonseminomatous germ cell tumors of the testis. AJR 1988; 151: 325-329. 2 Logothetis CJ, Samuels ML, Trindade A, Johnson DE. The growing teratoma syndrome. Cancer 1982; 50: 1629-1635. 3 Mostoti FK. Testicular tumors, epidemiology etiology and pathologic features. Cancer 1973; 32: 1186. 4 Wittes RE, Yagoda A, Silvay 0 et al.. Chemotherapy of germ cell tumors of the testis. Cancer 1976; 37: 637-645. 5 Glowalla M. Residual masses after successful chemotherapy for testicular carcinoma. AJR 1986; 146: 1184-l 186. 6 Stomper PC, Jochelson MS, Friedman EL et al. Evaluation of advanced seminoma treated with chemotherapy. AJR 1986; 745-748. 7 Stomper PC, Jochelson MS, Garnick MB, Richie JP. Residual abdominal masses after chemotherapy for nonseminomatous testicular cancer: correlation ofCI and histology. AJR 1985; 145: 743-746. 8 Hong WK, Wittes RE, Hajdu ST et al.. The evolution of mature teratoma from malignant testicular tumors. Cancer 1977; 40: 2987-2992. 9 Loehrer PJ, Mandelbaum I, Hui S et al. Resection of thoracic and abdominal teratoma in patients after cisplatin-based chemotherapy for germ cell tumor. J Thorac Cardiovasc Surg 1986; 92: 676-683.
