|Year : 2018 | Volume
| Issue : 10 | Page : 806-808
Renin-producing germ cell tumor in the pineal apparatus and mediastinum: A rare case report
Manabu Uematsu, Hiroshi Yokouchi, Yoshinori Tanino, Mitsuru Munakata
Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima 960-1295, Japan
|Date of Web Publication||24-Sep-2018|
Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960-1295
Source of Support: None, Conflict of Interest: None
Ectopic extrarenal renin-producing tumor is a rare disease with approximately 30 case reports in English literature. We herein present the first case of renin-producing germ cell tumors in the pineal apparatus and mediastinum. A 26-year-old man who had undergone craniotomy for the treatment of pineal tumor was found to have hypertension at a regular visit postoperatively. Laboratory findings revealed high plasma levels of renin activity and that of aldosterone concentration. Chest computed tomography demonstrated a large tumor in the mediastinum. The pathological findings revealed the mediastinal germ cell tumor positive for renin. The present case suggests that for young patients presenting with hypertension with a mediastinal tumor, the possibility of a renin-producing tumor should be considered.
Keywords: Germ cell tumor, mediastinal tumor, pineal apparatus, renin-producing tumor
|How to cite this article:|
Uematsu M, Yokouchi H, Tanino Y, Munakata M. Renin-producing germ cell tumor in the pineal apparatus and mediastinum: A rare case report. J Can Res Ther 2018;14, Suppl S3:806-8
|How to cite this URL:|
Uematsu M, Yokouchi H, Tanino Y, Munakata M. Renin-producing germ cell tumor in the pineal apparatus and mediastinum: A rare case report. J Can Res Ther [serial online] 2018 [cited 2022 May 28];14, Suppl S3:806-8. Available from: https://www.cancerjournal.net/text.asp?2018/14/10/806/180682
| > Introduction|| |
Ectopic renin-producing tumor is a rare disease yet should be considered as one of the differential diagnoses for hypertension. Extrarenal renin-producing tumor is a much rarer disease with approximately 30 case reports in English literature, and only two cases of germ cell tumor producing renin were reported., We herein present a young patient with hypertension after surgery of pineal tumor, revealing the first case of renin-producing germ cell tumors arising from the mediastinum.
| > Case Report|| |
A 26-year-old man was admitted to our hospital because of disturbed consciousness in January 2010. His brain computed tomography (CT) indicated a diagnosis of obstructive hydrocephalus by pineal tumor. He underwent craniotomy. Pathological examination of the surgical specimen revealed that the tumor was germinoma [Figure 1]a. He underwent whole-brain radiation (30 Gy) followed by three cycles of etoposide (100 mg/m2) and carboplatin (area under curve of 5 mg/ml/min). The patient demonstrated complete response (CR).
|Figure 1: (a and b) The pineal and mediastinal tumors (H and E, ×400), and (c and d) renin (×400). Renin staining is positive either in the cytoplasm or on the cell membrane of both tumor cells. Scale bars, 500 μm|
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When he had a regular visit in July 2012, he was found to have mild hypertension, at around 150/110 mmHg. Laboratory findings revealed high plasma levels of renin activity (PRA) (100 ng/ml/h; normal range, 0.3–2.9 ng/ml/h) and that of aldosterone concentration (491 pg/ml; normal range, 29.9–159 pg/ml). No abnormalities were found in the adrenal glands or kidneys on abdominal CT. However, chest CT showed a large tumor with a size of 135 mm × 112 mm in the mediastinum and a metastatic lesion in the left rib [Figure 2]a and [Figure 2]b. We conducted percutaneous ultrasound-guided transthoracic needle biopsy from the mediastinal tumor. Hematoxylin-eosin stain demonstrated large tumor cells with eosinophilic cytoplasm and with a large circular nucleus growing like cobblestones. The pathological diagnosis was seminoma [Figure 1]b. Given the increased serum level of alpha-fetoprotein (AFP) (254.9 ng/ml; normal range 0–10 ng/ml), we clinically diagnosed that the patient had a mixed germ cell tumor in the mediastinum.
|Figure 2: (a and b) Chest computed tomography shows a large tumor residing in the left mediastinum and the metastatic lesion in the left rib at diagnosis, and (c and d) those tumors after two cycles of chemotherapy|
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The patient underwent chemotherapy with bleomycin (10 mg/m2), etoposide (100 mg/m2), and cisplatin (20 mg/m2). After two cycles of the combined chemotherapy, the mediastinal tumor shrank significantly to the size of 65 mm × 58 mm [Figure 2]c with stable disease in rib metastasis [Figure 2]d. Furthermore, the serum levels of AFP and the PRA were reduced to 4.3 ng/ml and 0.2 ng/ml/h, respectively. Consistent with these findings, his blood pressure returned to the normal range. Immunohistochemistry using rabbit anti-renin polyclonal antibody (ab73447; Abcam, Cambridge, UK) demonstrated that the pineal and mediastinal tumors were both positive [Figure 1]c and [Figure 1]d. After the completion of four cycles of chemotherapy, the patient underwent open surgery for the resection of the tumor; however, he ended up into exploratory thoracotomy because the tumor had invaded the main trunk of the pulmonary artery. He received additional two courses of chemotherapy and has since remained in good health condition with no detectable recurrence at the time of writing (September 2015).
| > Discussion|| |
Renin-producing tumors are a rare disease. Approximately, 90 cases have been reported since 1967. Most of them are renal juxtaglomerular cell tumors. Extrarenal renin-producing tumor is a much rarer disease with approximately 30 case reports in English literature since 1977. This type of tumor has been reported to arise from carcinoma of the pituitary, orbit, lung, liver, adrenal gland, bladder, ovary, and testis. To the best of our knowledge, only two cases of ectopic renin-producing “germ cell tumors” (teratoma) derived from the ovary were reported., This is the first case of renin-producing germ cell tumors in the pineal apparatus and mediastinum.
The pathological diagnosis of the mediastinal tumor was consistent with that of a pineal tumor [Figure 1]a and [Figure 1]b. However, several reports support the idea that the two tumors in this case might be metachronous,, offering the following reasons: (i) Both organs have a predilection for germ cell tumors, (ii) extracranial metastasis of germinoma is rare, and (iii) over 2 years have elapsed since the first germ cell tumors occurred in pineal apparatus, which achieved CR.
Primitive germ cells arise in the 3rd week of embryonic life and then move to the gonadal ridge in the 5th to 6th week. In the interim period (from the 3rd to the 5th week), primitive germ cells acquire pluripotency under certain conditions including the production of AFP. Extragonadal germ cell tumors are believed to arise by migrating to the favorite sites such as the pineal apparatus and mediastinum between the 5th and 6th week. Based on the aforementioned principles, germ cells with potentiation of renin production might reasonably arise from the 3rd to the 5th week and then migrate to other organs through unknown mechanisms. To the best of our knowledge, no reports have demonstrated molecules displaying ectopic renin production. Recently, potassium channel, inwardly rectifying subfamily J, member 5 germline mutation was reported to be associated with the development of primary hyperaldosteronism. Such genetic changes might have altered the character of the embryo in our case, leading to spatiotemporally aberrant renin production. Therefore, we should carefully monitor this patient if another germ cell tumor with renin production arises in the future.
Left rib metastasis was not reduced after two to six cycles of chemotherapy; however, a 18F-fluorodeoxyglucose positron emission tomography scan after four cycles of chemotherapy showed a remarkable signal reduction, suggesting a good response to the treatment. We referred the patient to an orthopedist in our hospital at this time. Based on the results of the PET scan and the low density on CT, which suggested necrosis, the orthopedist agreed with our diagnosis and recommended avoiding a needle biopsy. A previous review article demonstrated that cancer cells affect the distribution of both osteoclasts and osteoblasts in bone and that the balance of both cells determines either osteolytic or osteoplastic change. In some cases, augmented osteoblasts occupy the space in the bone and maintain the size of the bone metastasis after treatment. In our case, CT after two cycles of chemotherapy showed calcification in the margin of the rib metastasis [Figure 2]d, indicating osteoplastic change. This calcification might have affected the clearance of necrotic tumor tissue.
The present case suggests that for young patients presenting with hypertension with a mediastinal tumor, the possibility of a renin-producing tumor should be considered. Further investigation of the development of renin-producing germ cell tumor is warranted.
The authors thank Dr. Kenichi Tanaka (Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolism Medicine, Fukushima Medical University School of Medicine), Dr. Mizuko Tanaka (Department of Basic Pathology, Fukushima Medical University School of Medicine), and Dr. Masahiro Ichikawa (Department of Neurosurgery, Fukushima Medical University School of Medicine) for their contributions to this manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]