882 CASE REPORTS

differentiation, such as that described for gastrointestinal adenocarcinoid (6,7).

In this case, cisplatin-based chemotherapy was not effec- tive, but radiation therapy was slightly effective. In general, radiation therapy is occasionally helpful in the treatment of thymic carcinoid tumours causing superior vena cava obstruction. There is scant information regarding chemo- therapy of thymic carcinoid and the precise role of chemo- therapy is unclear. Additional exploration of chemotherapy in this disease is needed.

References

1. Herbst WM, Kummer W, Hofmann W, Otto, H, Heym C. Carcinoid tumors of thymus. An imunohistochemical study. Cancer 1987; 60: 2465-2470.

2. Wick MR, Rosai J. Neuroendocrine neoplasms of the thymus. Path01 Res Pratt 1988; 183: 188-199.

3. Rosai J, Levine G, Weber WR, Higa E. Carcinoid tumors and oat cell carcinoma of the thymus. Path01 Ann 1976; 11: 201-226.

4. Rosai J, Higa E. Mediastinal endocrine neoplasm, of probably thymic origin, related to carcinoid tumor. Clinicopathologic study of 8 cases. Cancer 1972; 29: 1061-1074.

5. Suster S, Cesar AM. Thymic carcinoid with prominent mutinous stroma. Report of a distinctive morphologic variant of thymic neuroendocrine neoplasm. Am J Surg Pathol 1995; 19: 1277-1285.

6. Warkel RL, Cooper PH, Helwig EB. Adenocarcinoid, a mucin producing carcinoid tumor of the appendix. Cancer 1978; 42: 2781-2793.

7. Subbuswamy SG, Gibbs NM, Ross CF, Morson BC. Goblet cell carcinoid of the appendix. Cancer 1974; 34: 338-344.

Primary mediastinal nonseminomatous germ cell tumour in an adult female

Y. MORISHIMA~, H. SATOH”, M. OHTSUKA~, T. YAZAWA+, Y. YAMASHITA* AND S. HASEGAWA”

“Pulmonary Division, Department of Internal Medicine, Institute of Clinical Medicine, University of Tsukuba, ]apan ‘Department of Pathology, Institute of Basic Medical Science, University of Tsukuba, Japan

Introduction

Primary mediastinal nonseminomatous germ cell tumours (MNGCTs) are highly malignant and extremely rare in females (1). As far as we reviewed the literature written in English through MEDLINE, only 33 female cases with MNGCTs have been reported since 1952. It has been suggested that cisplatin-based chemotherapy improves out- come of the patients with MNGCTs; however, the experi- ence in female patients is limited (14). In this article, we report a young female with MNGCT who was diagnosed by the careful evaluation of computed tomographic find- ings, biopsy results and serological tumour markers, and successfully treated with cytoreductive surgery and intensive cisplatin-based chemotherapy.

Received 12 December 1996 and accepted 20 February 1997. Correspondence should be addressed to: H. Satoh, Pulmonary Division, Department of Internal Medicine, Institute of Clinical Medicine, University of Tsukuba, Tsukuba-city, Ibaraki 3054575, Japan.

Case Report

An 18 year old college student was found to have a mediastinal mass on a screening chest radiograph required for her college admission. She was totally asymptomatic with stable vital signs. Physical examinations only revealed a mild jugular venous distention. Chest computed tomogra- phy (CT) scan showed an 8-cm right-sided anterior medi- astinal mass that was mainly composed of multilocular cysts with fat density, and a solid part was present in the anterior portion. Posteriorly, the mass displaced media- stinal structures, obliterated the fat planes of the media- stinum, and contrast-enhanced borders of the mass compressed the right lung. The mass also abutted against the chest wall without invasion. Neither intrapulmonary nodules nor lymphadenopathy was observed (Fig. 1).

Under ultrasound and roentgenoscopic guidance, a fine needle aspiration biopsy was performed towards the cystic area, whereas the solid part was approached by a 14-gauge tru-cut biopsy needle. Cystic space contained dark brown liquid and cytology revealed a mature teratoma. However,

CASE REPORTS 883

FIG. 1. Chest CT scan film showing a large cystic mass in the right anterior mediastinum. A solid part is present in the anterior portion.

FIG. 2. Histological finding of resected mass showing a mixture of several neoplastic components, such as embryonal carcinoma and endodermal sinus tumour.

the solid part was suspected as embryonal carcinoma. The serum concentration of a fetoprotein (AFP) was elevated to 9370 ng ml ~ I, whereas the level of human chorionic gona- dotropin (HCG) was within normal limits. The AFP level obtained from fluid in the cystic space was 2159 ng ml - I. Otherwise, there were no abnormal findings suggestive of metastases by abdominal ultrasound, head CT scan or bone scintigram.

A mediastinotomy was performed and an 11 x 8.5 x 9 cm mass was removed en bloc with the part of the right upper and middle lobes as well as thymus. The tumour was well encapsulated and contained both cystic and solid parts as seen in the CT scan. Microscopic observations revealed that the cyst wall was partly lined with epidermal epi- thelium. The solid part was composed of a mixture of mature and immature teratoma as well as embryonal carcinoma and endodermal sinus tumour, and most of the neoplastic lesions showed necrotic changes. Those find- ings were all consistent with the diagnosis of MNGCT. There was no extension of tumour cells outside the capsules (Fig. 2).

Surgery was uneventful and she had a stable postopera- tive course. The serum level of AFP declined exponentially

with a half-life of 5.5 days. A repeat chest CT scan obtained on postoperative day 21, however, revealed a total of five new nodular lesions, which suggested metastases, in both lungs. The intensive chemotherapy with cisplatin (90mgm-‘, day l), vinblastine (0.2 mg kg- ‘, day 1, 2) and bleomycin (12 mg rn-‘, day 2) was begun on post- operative day 23 and six courses were given, every 3rd or 4th week.

During the chemotherapy, she developed severe emesis, paralytic ileus, jaw pain and neutropenic fever for which she received granulocyte-colony stimulating factor, antibiotic treatment and vigorous supportive care. The nodular lesions disappeared after the first course of chemotherapy. The serum AFP levels declined in a logarithmic fashion and became undetectable at the end of the 2nd cycle of chemo- therapy. She tolerated the intensive chemotherapy and has been well, without any more side-effects, 29 months after the diagnosis was made. She is now enjoying her school life without evidence of local recurrence or metastases.

Discussion

Mediastinal germ cell tumours represent approximately 15% of anterior mediastinal tumours in adults. The most common histological type is mature teratoma followed by seminoma, and MNGCTs are less common. More than 90% of malignant germ cell tumours in the mediastinum occur in men. Because the therapeutic approaches and the prognosis are largely dependent on the pathological ciassi- fication, early diagnosis of MNGCTs is important (5). Chest CT scans of MNGCTs are characteristic with find- ings of large masses with heterogeneous attenuation. The masses often contain large central lesions of near-water density indicating cyst formation, haemorrhage or necrosis (6,7). Fat planes are often obliterated, and the interface between the tumour and the adjacent lung may be irregular and spiculated due to lung invasion. Seminomas usually demonstrate large and lobulated masses with homogeneous soft-tissue attenuation on CT. Mature teratomas typically show several attenuation values: calcium, fat, and fluid in the masses, sharply marginate against the adjacent lung. Thymomas and thymic carcinoid tumours have appear- ances similar to those of seminomas. However, when thymomas undergo cystic degeneration and include calcifi- cation, the differentiation from MNGCTs may be difficult. Anterior mediastinal lymph nodes and/or thymic infiltra- tion of Hodgkin’s disease with a necrotic degeneration may also resemble MNGCTs. Thymic cysts are well-defined homogeneous masses with water density, and the walls of cysts are thin and smooth.

Cisplatin-based chemotherapy, especially the combina- tion of cisplatin, vinblastine and bleomycin (PVB), which were originally used to treat gonadal germ cell tumours with favorable outcome, have achieved complete remission in above 50% of patients with MNGCTs, and approxi- mately 50% of them are expected to be long-term survivors (5). Furthermore, Vogelzang et al. suggested that therapy with cisplatin, etoposide, bleomycin, and doxorubicin (CEBA regimen) is more effective than standard PVB

884 CASE REPORTS

regimen in the management of MNGCTs (8). Williams et al. also recommended the superiority of therapy with cisplatin, etoposide and bleomycin compared to PVB regi- men in the treatment of advanced disseminated germ cell tumours (9). However, several investigators have recently reported that etoposide-containing chemotherapy increased risk of leukemia when used to treat germ cell tumours (10,ll). Therefore, we chose PVB regimen rather than CEBA regimen, although MNGCTs themselves may have a potential risk in development of haematological malignancies.

We feel that a needle biopsy following a careful evalu- ation of CT findings and serological markers will be a rapid, safe, and unstressful procedure in differentiating MNGCTs from other mediastinal tumours in adult females, and that combination of cytoreductive surgery and intensive chemotherapy with cisplatin-based regimen is effective in cases of MNGCTs.

Acknowledgements

The authors thank Drs Michio Kaneko, Kiyofumi Mitsui and Takesaburo Ogata for advice.

References

1. Nichols CR, Saxman S, Williams SD et al. Primary mediastinal nonseminomatous germ cell tumors. Cancer 1990; 65: 1641-1646.

2. Knapp RH, Hurt RD, Payne WS et al. Malignant germ cell tumors of the mediastinum. J Thorac Cardiovasc Surg 1985; 89: 82-89.

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Gooneratne S, Keh P, Sreekanth S, Recant W, Talerman A. Anterior mediastinal endodermal sinus (York sac) tumor in a female infant. Cancer 1985; 56: 1430-1433. LemariC E, Assouline PS, Diot P et al. Primary mediastinal germ cell tumors. Results of a French retrospective study. Chest 1992; 102: 1477-1483. Nichols CR. Mediastinal germ cell tumors: clinical features and biologic correlates. Chest 1991; 99: 472- 479. Lee KS, Im J, Han CH, Han MC, Kim C, Kim WS. Malignant primary germ cell tumors of the media- stinum: CT features. AJR 1989; 153: 947-951. Rosado-de-Christenson ML, Templeton PA, Moran CA. Mediastinal germ cell tumors: radiologic and pathologic correlation. Radiographics 1992; 12: 1013- 1030. Vogelzang NJ, Anderson RW, Kennedy BJ. Successful treatment of mediastinal germ cell/endodermal sinus tumors. Chest 1985; 88: 64-69. Williams SD, Birch R, Einhorn LH, Irwin L, Greco FA, Loehrer PJ. Treatment of disseminated germ-cell tumors with cisplatin, bleomycin, and either vincristine or etoposide. N Engl J &fed 1987; 316: 1435-1440. Pedersen-Bjergaard J, Daugaard G, Hansen SW, Philip P, Larsen SO, Rerrth M. Increased risk of myelodys- plasia and leukaemia after etoposide, cisplatin, and bleomycin for germ cell tumours. Lancet 1991; 338: 359-363. Boshoff C, Begent RHJ, Oliver RTD et al. Secondary tumors following etoposide containing therapy for germ cell cancer. Ann Oncol 1995; 6: 3540.

Idiopathic bronchiolitis obliterans with organizing pneumonia presenting with adult respiratory distress syndrome

L. A. I%REZ DE LLANO”, J. L. SOILAN”, M. J. GARCIA PAIS”, I. MATA+, M. MOREDA* AND B. LASERNA’

“Respiratory Division, ‘Department of Pathology, *Department of Swgevy, and ‘Department of Anesthesiology, Hospital Xeval-Calde, Lugo, Spain

Introduction pneumonia, is a clinicopathological syndrome that has emerged in the past decade as an important cause of

Idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP), also known as cryptogenic organizing

respiratory illness in adults (1,2). Clinically, patients with this syndrome have been reported to present with a short prodrome characterized by a viral-type illness, followed by

Received 7 September 1996 and accepted 27 February 1997. Correspondence should be addressed to: L. A. PBrez de Llano, Respiratory Division, Hospital Xeral-Calde, c/Dr. Ochoa s/n, 27004 Lugo, Spain.

cough, dyspnea and constitutional symptoms. Organized inflammatory polipoid masses predominantly affecting distal bronchioles and alveolar ducts are the major patho- logical findings. The course of this entity has usually been