CASE REPORTS Systemic Mast Cell Disease Associated with Primary Mediastinal Germ Cell Tumor PATRICK CHARIOT, M.D., ISABELLE MONNET, M.D., FRAN(;OISE LELONG, M.D., CLAUDE CHLEQ, M.D., Cr~teil, France, JEAN-PIERRE DROZ, M.D., Villejuif, France, HUBERT de CREMOUX, M.D., Cr~teil, France The f'~st known case of systemic mast cell dis- ease associated with a germ cell tumor is report- ed. Six months after the complete remission of a primary media~tlnR! germ cell tumor treated by chemotherapy and resection, a young man had a series of episodes of hypotension and syncope and, a few months later, a gastric hemorrhage and coagulation disorder. The diagnosis of sys- temic ma~t cell disease was made in view of bone m~rrow and liver biopsies. A tran.~ient circulat- ing heparin-like anticoagulant was noted. The occurrence in a short period of these two unusu- al conditions, given previous knowledge of he- matologic disorders associated with germ cell tumors, suggests that the present association is not a coincidence. Systemic ma~t cell disease should be considered among the hematologic dis- orders associated with a germ cell tumor. From the Departments of Pneumology,Hematology, and P~thology, Centre Hospitalier Intercommunal de Cr6teil, Cr~teil, France, and the Department of Medical Oncology, Institute Gustave-Roussy, Villejuif, France. Requests for reprints should be addressedto IsabelleMonnet, M.D., Clinique de Pathologie Respiratoire, Centre Hospitalier Intercommunal, 94000 Cr~teil, France. Manuscript submitted March 2, ]990, and accepted in revised form July 12, 1990. G erm cell tumors are uncommon malignancies that primarily affect the testes in young men. About 2% of germ cell tumors arise in extragonadal sites, most of them in the mediastinum [1]. Systemic mast cell disease (SMCD) is character- ized by abnormal proliferation of mast cells in vari- ous tissues; infiltration of bone marrow, skin, liver, spleen, and lymph nodes may occur [2,3]. The origin of this rare process remains unknown. Its associa- tion with hematologic disorders and solid malig- nancies has been previously described [3,4]. Recent reports mentioned the development of hematologic malignancies in patients with medias- tinal germ cell tumors [5-11], but the association of SMCD with a germ cell tumor has not been report- ed so far. The present case is also remarkable for the rare occurrence of a circulating heparin-like anticoagulant. CASE REPORT A 34-year-old man was referred to our hospital in December 1987 for acute dyspnea; clinical and roentgenologic examination revealed pericardial tamponade and a left anterior mediastinal mass. Biopsy material showed embryonal carcinoma without any mature components. The human chori- onic gonadotropin (HCG) level was normal, but the ~-fetoprotein level was high at 433 ng/mL (normal: less than 20 ng/mL). No extrathoracic involvement was found and the diagnosis of primary mediastinal germ cell tumor was established. A bone marrow examination was not done. The patient was given four cycles of chemotherapy with cisplatin, bleomy- cin, etoposide, and vinblastine. His a-fetoprotein level normalized but the mediastinal mass showed incomplete regression. In April 1988, the patient underwent left thoracotomy with removal of the residual mass. Histology showed a predominant mature teratoma with sparse malignant cells. On account of persistent immature elements, two more cycles of chemotherapy with etoposide and ifosfa- mide were given (June 1988 to July 1988). The pa- tient was believed to be in complete remission. In January 1989, he presented with the first epi- sode of flushing, hypotension, tachycardia, and di- arrhea. Several similar attacks occurred in the fol- March 1991 The American Journal of Medicine Volume90 381 ..