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An unusual mediastinal tumor in a patient with aids
BRIEF CLINICAL
In an attempt to clarify the confusion in the literature, we propose that vascular compression of the duodenum between an aortic aneurysm and the superior mesenteric artery be classified as one of the many causes of superior mesenteric artery syndrome. An aortic aneurysm can &use narrowing of the angle between the superior mesenteric artery and its point of origin from the aorta, thus predisposing to “vascular compression” of the duodenum. Therefore, “vascular compression of the duodenum” is a subcategory of superior mesenteric syndrome and not a separate entity. In reviewing the 16 previously reported cases of duodenal obstruction by an abdominal aortic aneurysm [l-5], we found most patients presented with nausea, vomiting, and weight loss, and had an abdominal mass on physical examination. Four patients did well after gastrojejunostomy or duodenojejunostomy. Only five previously reported patient8 have survived an aneurysmal repair [3-51. Our patient is the oldest patient surviving surgery in this setting. Ideally, the treatment of choice is aneurysm resection. However, appreciable morbidity and mortality are associated with this surgical procedure in this age group, and, therefore, relieving the obstruction with intestinal bypass may be preferred.
OBSERVATIONS
AN UNUSUAL MEDIASTINAL TUMOR IN A PATIENT WITH AIDS An association between infection with human im-
munodeficiency virus (HIV) and some malignancies, particularly Kaposi’s sarcoma or non-Hodgkin’s lymphoma, has been defined. An association of testicular germ cell tumor8 with HIV disease has been noted by 8ome and raise8 the possibility that these tumor8 are more prominent in these patients [l-4]. We present a patient with acquired immunodeficiency syndrome (AIDS) and a primary mediastinal mixed germ cell tumor. A 39-year-old man with a history of homosexuality and intravenous drug abuse was found to be HIVseropositive in December 1990 when he developed fieumocystis carinii pneumonia. At that time his CD4 count wa8 102; on discharge, he began to receive zidovudine and aerosolized pentamidine. He did well until January 1992, when he developed a cough with occasional blood-tinged sputum, fever, night sweats, mild dyspnea, and generalized malaise. Result8 of physical examination were significant for a mobile and nontender 2 X 3-cm right scalene lymph node without other adenopathy. His lungs were clear. His liver was at the right costal margin and nontender, and result8 of testicular examination were normal. His complete blood count and chemistry 18 profile were remarkable only for a MARK SOSTEK,M.D. STEVENN. FINE, M.D. platelet count of 81,000, aspartate aminotransferase level of 203 U/L, and a lactate dehydrogenase TRACYL. HARRIS,IO. Lahey Clinic Medical Center level of 773 U/L. Hi8 chest radiograph revealed Burlington, Massachusetts marked widening of the anterior superior mediastinum (Figure 1). Computed tomography (CT) of the neck and thorax revealed a large 10 cm X 10 cm 1. Hodgson KJ, Webster DJ. Abdominal aortic aneurysm causing duodenal and X 20 cm anterior mediastinal mass and a 2 cm X 3 ureteric obstructfon. J Vast Surg 1986; 3: 364-8. 2Adair HM. Duodenal obstruction due to abdominal aortic aneurysm. BMJ cm X 3 cm right anterior scalene node, with associ1975; 2: 727. ated subcarinal and left hilar adenopathy, causing 3. Hough DR. O’Meara TF. Abdominal aortic aneurysm with initial symptom of compromise of the left main-stem bronchus (Figduodenal obstruction. Am J Gastroenterol 1981:76:538-41. ure 2). CT of the abdomen and pelvis demonstrated 4. Edwards KC, Katzen BT. Superior mesenteric artery syndrome due to large dissectfng abdominal aortic aneurysm. Am J Gastrcenterol 1984; 79: 724. hepatosplenomegaly, several low-density areas in 5. Coster DD, Stubbs DH, Sidney DT. Duodenal obstruction by abdominal aortic the lateral aspect of the spleen after contrast, and aneurysms. Am J Gastroenterol 1988; 83: 981-4. no abdominal or pelvic lymphadenopathy. 6. Hines J, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Biopsy of the right supraclavicular mass demonDiiic criteria and therapeutic approaches. Am J Surg 1984; 148: 630-2. 7. Akin JT Jr, Gray SW, Skandafakis JE. Vascular compression of the duodenum: strated a grossly necrotic mass that was 3 to 4 cm presentation of ten cases and review of the literature. Surgery 1976; 79: deep. Histopathology revealed viable areas of se515-22. minoma rimming blood vessels. No other germ cell 8. Gondos B. Duodenal compression defect and the “superior mesenteric artery component8 were identified. The tumor demonsyndrome” 1. Radii 1977; 123: 575-80. 9. Lee CS. Mangle JC. Superior mesenteric artery compression syndrome. Am J strated a high mitotic rate, with three or more mitoGastroenterol 1978; 70: 141-50. ses per high-power field. The patient’s /3-human 10. Nugent FW. Braasch JW. Epstein H. Diagnosis and surgfcal treatment of chorionic gonadotropin (@-HCG) level was 5.4 arteriomesenteric obstruction of the duodenum. JAMA 1966; 196: 10913. mIU/mL (normal less than 5 mIU/mL), and the (Y11.von Rokitansky CF. Lehrbuch der pathologischen Anatomie. Ed. 1. Wein: WB Raumuller, 1861. fetoprotein (AFP) level was 777.5 ng/mL (normal 12.Akin JT. The anatomic basis of vascular compression of the duodenum. Surg less than 15 ng/mL). These results were consistent Clin North Am 1974:54: 1361-9. with a mixed germ cell tumor. The patient underwent one cycle of chemotherapy with platinum, VP-16, and bleomycin. The paSubmitted August 28, 1992, and accepted October 1. 1992 February
1993
The American
Journal
oi Medicine
Volume
94
221
BRIEF CLINICAL
OBSERVATIONS
Figure
2. CT scan of the chest
showing
a large anterior
medi-
astinal mass.
Figure
1. Chest
radiograph
showing
widening
of the mediasti-
num.
tient died 2 weeks later without appreciable clinical response. No postmortem examination was obtained. Malignancies have been recognized in HIV infection since the pandemic began. The two AIDS-defining malignancies, Kaposi’s sarcoma and nonHodgkin’s lymphoma, clearly occur more commonly in patients infected with HIV. The occurrence of other solid tumors in patients with HIV infection may simply represent a chance occurrence or may result from impaired immune surveillance due to an alteration in the immune system due to HIV [5]. There is growing evidence that the incidence of testicular tumors in HIV-positive patients exceeds that of the general male population. Wilson et al [6] reported an incidence of 0.2% (5 HIV-seropositive patients with 6 testicular tumors out of a group of 3,015 patients seen at a hospital-based AIDS clinic over 2 years) in comparison to the United States average of 3.5 new cases per 100,000 male patients per year. A retrospective analysis by Tessler and Cantanese [4] in 1987 demonstrated that 25 of 115 patients with testicular tumors were found to have AIDS or AIDS-related complex (ARC), or to be at risk for the development of AIDS. Patients with
222
February
1993
The Amerlcen
Journal
of Medicine
Volume
AIDS did poorly with therapy compared with patients with ARC or HIV seropositivity alone. Further epidemiologic studies are needed to evaluate this relationship. We searched Grateful Med, Aidsline, and Cancerline using the key words HIV, AIDS, seminoma, mediastinal tumors, and extragonadal germ cell tumors and found no previous report of primary mediastinal germ cell tumor in an HIV-infected individual. This case highlights the need for pursuit of specific tissue diagnosis in HIV patients with mediastinal masses and cervical lymphadenopathy. It also suggests the potential value for measurement of both &HCG and AFP levels in AIDS patients with mediastinal masses. Our patient did poorly with therapy. Further investigation is necessary to define which patients with advanced germ cell tumors and HIV infections might benefit from systemic therapy. VALERIEC. ROHLMAN,M.D. VIKKIA. CANFIELD,M.D. RONALDA. GREENFZELD,M.D.
University of Oklahoma Health Sciences Center Oklahoma City, Oklahoma 1. Adjiman S, Zerbib M, Flam T. et
al.Genitourinary
tumors and HIV 1 infection.
Eur Urol 1990; 18: 61-3. 2. Wilkinson M, Carroll PR. Testicular carcinoma
in patients positive and at risk
for human immunodeffciency virus. J Urol 1990; 144: 1157-9. 3. Palmer MC, Mador DR, Venner PM. Testicular seminoma associated with the acquired immunodeficiency syndrome and acquired immunodeficiency syndrome related complex: 2 case reports. J Urol 1989; 142: 126-30. 4. Tessler AN, Cantanese A. AIDS and germ cell tumors of testis. Urology 1987; 30: 203-4. 5. Kaplan MH, Susin M, Pahwa SG. et al. Neoplastic complications of HTLV Ill infections. Lymphomas and solid tumors. Am J Med 1987; 82: 389-96. 6. Wilson WT. Frenkel E, Vuitch F, Sagalowsky Al. Testicular tumors in men with human immunodeficiency virus. J Urol 1992; 147: 1036-40. Submitted
94
July 14, 1992, and accepted
October
12. 1992
In an attempt to clarify the confusion in the literature, we propose that vascular compression of the duodenum between an aortic aneurysm and the superior mesenteric artery be classified as one of the many causes of superior mesenteric artery syndrome. An aortic aneurysm can &use narrowing of the angle between the superior mesenteric artery and its point of origin from the aorta, thus predisposing to “vascular compression” of the duodenum. Therefore, “vascular compression of the duodenum” is a subcategory of superior mesenteric syndrome and not a separate entity. In reviewing the 16 previously reported cases of duodenal obstruction by an abdominal aortic aneurysm [l-5], we found most patients presented with nausea, vomiting, and weight loss, and had an abdominal mass on physical examination. Four patients did well after gastrojejunostomy or duodenojejunostomy. Only five previously reported patient8 have survived an aneurysmal repair [3-51. Our patient is the oldest patient surviving surgery in this setting. Ideally, the treatment of choice is aneurysm resection. However, appreciable morbidity and mortality are associated with this surgical procedure in this age group, and, therefore, relieving the obstruction with intestinal bypass may be preferred.
OBSERVATIONS
AN UNUSUAL MEDIASTINAL TUMOR IN A PATIENT WITH AIDS An association between infection with human im-
munodeficiency virus (HIV) and some malignancies, particularly Kaposi’s sarcoma or non-Hodgkin’s lymphoma, has been defined. An association of testicular germ cell tumor8 with HIV disease has been noted by 8ome and raise8 the possibility that these tumor8 are more prominent in these patients [l-4]. We present a patient with acquired immunodeficiency syndrome (AIDS) and a primary mediastinal mixed germ cell tumor. A 39-year-old man with a history of homosexuality and intravenous drug abuse was found to be HIVseropositive in December 1990 when he developed fieumocystis carinii pneumonia. At that time his CD4 count wa8 102; on discharge, he began to receive zidovudine and aerosolized pentamidine. He did well until January 1992, when he developed a cough with occasional blood-tinged sputum, fever, night sweats, mild dyspnea, and generalized malaise. Result8 of physical examination were significant for a mobile and nontender 2 X 3-cm right scalene lymph node without other adenopathy. His lungs were clear. His liver was at the right costal margin and nontender, and result8 of testicular examination were normal. His complete blood count and chemistry 18 profile were remarkable only for a MARK SOSTEK,M.D. STEVENN. FINE, M.D. platelet count of 81,000, aspartate aminotransferase level of 203 U/L, and a lactate dehydrogenase TRACYL. HARRIS,IO. Lahey Clinic Medical Center level of 773 U/L. Hi8 chest radiograph revealed Burlington, Massachusetts marked widening of the anterior superior mediastinum (Figure 1). Computed tomography (CT) of the neck and thorax revealed a large 10 cm X 10 cm 1. Hodgson KJ, Webster DJ. Abdominal aortic aneurysm causing duodenal and X 20 cm anterior mediastinal mass and a 2 cm X 3 ureteric obstructfon. J Vast Surg 1986; 3: 364-8. 2Adair HM. Duodenal obstruction due to abdominal aortic aneurysm. BMJ cm X 3 cm right anterior scalene node, with associ1975; 2: 727. ated subcarinal and left hilar adenopathy, causing 3. Hough DR. O’Meara TF. Abdominal aortic aneurysm with initial symptom of compromise of the left main-stem bronchus (Figduodenal obstruction. Am J Gastroenterol 1981:76:538-41. ure 2). CT of the abdomen and pelvis demonstrated 4. Edwards KC, Katzen BT. Superior mesenteric artery syndrome due to large dissectfng abdominal aortic aneurysm. Am J Gastrcenterol 1984; 79: 724. hepatosplenomegaly, several low-density areas in 5. Coster DD, Stubbs DH, Sidney DT. Duodenal obstruction by abdominal aortic the lateral aspect of the spleen after contrast, and aneurysms. Am J Gastroenterol 1988; 83: 981-4. no abdominal or pelvic lymphadenopathy. 6. Hines J, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Biopsy of the right supraclavicular mass demonDiiic criteria and therapeutic approaches. Am J Surg 1984; 148: 630-2. 7. Akin JT Jr, Gray SW, Skandafakis JE. Vascular compression of the duodenum: strated a grossly necrotic mass that was 3 to 4 cm presentation of ten cases and review of the literature. Surgery 1976; 79: deep. Histopathology revealed viable areas of se515-22. minoma rimming blood vessels. No other germ cell 8. Gondos B. Duodenal compression defect and the “superior mesenteric artery component8 were identified. The tumor demonsyndrome” 1. Radii 1977; 123: 575-80. 9. Lee CS. Mangle JC. Superior mesenteric artery compression syndrome. Am J strated a high mitotic rate, with three or more mitoGastroenterol 1978; 70: 141-50. ses per high-power field. The patient’s /3-human 10. Nugent FW. Braasch JW. Epstein H. Diagnosis and surgfcal treatment of chorionic gonadotropin (@-HCG) level was 5.4 arteriomesenteric obstruction of the duodenum. JAMA 1966; 196: 10913. mIU/mL (normal less than 5 mIU/mL), and the (Y11.von Rokitansky CF. Lehrbuch der pathologischen Anatomie. Ed. 1. Wein: WB Raumuller, 1861. fetoprotein (AFP) level was 777.5 ng/mL (normal 12.Akin JT. The anatomic basis of vascular compression of the duodenum. Surg less than 15 ng/mL). These results were consistent Clin North Am 1974:54: 1361-9. with a mixed germ cell tumor. The patient underwent one cycle of chemotherapy with platinum, VP-16, and bleomycin. The paSubmitted August 28, 1992, and accepted October 1. 1992 February
1993
The American
Journal
oi Medicine
Volume
94
221
BRIEF CLINICAL
OBSERVATIONS
Figure
2. CT scan of the chest
showing
a large anterior
medi-
astinal mass.
Figure
1. Chest
radiograph
showing
widening
of the mediasti-
num.
tient died 2 weeks later without appreciable clinical response. No postmortem examination was obtained. Malignancies have been recognized in HIV infection since the pandemic began. The two AIDS-defining malignancies, Kaposi’s sarcoma and nonHodgkin’s lymphoma, clearly occur more commonly in patients infected with HIV. The occurrence of other solid tumors in patients with HIV infection may simply represent a chance occurrence or may result from impaired immune surveillance due to an alteration in the immune system due to HIV [5]. There is growing evidence that the incidence of testicular tumors in HIV-positive patients exceeds that of the general male population. Wilson et al [6] reported an incidence of 0.2% (5 HIV-seropositive patients with 6 testicular tumors out of a group of 3,015 patients seen at a hospital-based AIDS clinic over 2 years) in comparison to the United States average of 3.5 new cases per 100,000 male patients per year. A retrospective analysis by Tessler and Cantanese [4] in 1987 demonstrated that 25 of 115 patients with testicular tumors were found to have AIDS or AIDS-related complex (ARC), or to be at risk for the development of AIDS. Patients with
222
February
1993
The Amerlcen
Journal
of Medicine
Volume
AIDS did poorly with therapy compared with patients with ARC or HIV seropositivity alone. Further epidemiologic studies are needed to evaluate this relationship. We searched Grateful Med, Aidsline, and Cancerline using the key words HIV, AIDS, seminoma, mediastinal tumors, and extragonadal germ cell tumors and found no previous report of primary mediastinal germ cell tumor in an HIV-infected individual. This case highlights the need for pursuit of specific tissue diagnosis in HIV patients with mediastinal masses and cervical lymphadenopathy. It also suggests the potential value for measurement of both &HCG and AFP levels in AIDS patients with mediastinal masses. Our patient did poorly with therapy. Further investigation is necessary to define which patients with advanced germ cell tumors and HIV infections might benefit from systemic therapy. VALERIEC. ROHLMAN,M.D. VIKKIA. CANFIELD,M.D. RONALDA. GREENFZELD,M.D.
University of Oklahoma Health Sciences Center Oklahoma City, Oklahoma 1. Adjiman S, Zerbib M, Flam T. et
al.Genitourinary
tumors and HIV 1 infection.
Eur Urol 1990; 18: 61-3. 2. Wilkinson M, Carroll PR. Testicular carcinoma
in patients positive and at risk
for human immunodeffciency virus. J Urol 1990; 144: 1157-9. 3. Palmer MC, Mador DR, Venner PM. Testicular seminoma associated with the acquired immunodeficiency syndrome and acquired immunodeficiency syndrome related complex: 2 case reports. J Urol 1989; 142: 126-30. 4. Tessler AN, Cantanese A. AIDS and germ cell tumors of testis. Urology 1987; 30: 203-4. 5. Kaplan MH, Susin M, Pahwa SG. et al. Neoplastic complications of HTLV Ill infections. Lymphomas and solid tumors. Am J Med 1987; 82: 389-96. 6. Wilson WT. Frenkel E, Vuitch F, Sagalowsky Al. Testicular tumors in men with human immunodeficiency virus. J Urol 1992; 147: 1036-40. Submitted
94
July 14, 1992, and accepted
October
12. 1992