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CORRESPONDENCE
Year : 2018  |  Volume : 14  |  Issue : 10  |  Page : 806-808

Renin-producing germ cell tumor in the pineal apparatus and mediastinum: A rare case report


Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima 960-1295, Japan

Date of Web Publication24-Sep-2018

Correspondence Address:
Hiroshi Yokouchi
Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960-1295
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.180682

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 > Abstract 


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 2020 Oct 26];14:806-8. Available from: https://www.cancerjournal.net/text.asp?2018/14/10/806/180682




 > Introduction Top


Ectopic renin-producing tumor is a rare disease yet should be considered as one of the differential diagnoses for hypertension.[1] Extrarenal renin-producing tumor is a much rarer disease with approximately 30 case reports in English literature,[2] and only two cases of germ cell tumor producing renin were reported.[3],[4] 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 Top


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 Top


Renin-producing tumors are a rare disease. Approximately, 90 cases have been reported since 1967.[5] 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.[2] 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.[3],[4] 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,[6],[7] 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[8] including the production of AFP.[9] 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.[10] 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[11] 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.

Acknowledgments

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Gupta V. Mineralcorticoid hypertension. Indian J Endocrinol Metab 2011;15 Supple 4:S298-312.  Back to cited text no. 1
    
2.
Ohmori H, Motoi M, Sato H, Tsutsumi A, Ogawa K. Extrarenal renin-secreting tumor associated with hypertension. Acta Pathol Jpn 1977;27:567-86.  Back to cited text no. 2
    
3.
Pursell RN, Quinlan PM. Secondary hypertension due to a renin-producing teratoma. Am J Hypertens 2003;16:592-5.  Back to cited text no. 3
    
4.
Matsuyama S, Yamamoto Y, Sakate S, Hirata E, Fujiwara H, Kudo Y. A case of renin-producing ovarian teratoma with malignant transformation. Mod Trends Obstet Gynecol 2011;60:395-9.  Back to cited text no. 4
    
5.
Robertson PW, Klidjian A, Harding LK, Walters G, Lee MR, Robb-Smith AH. Hypertension due to a renin-secreting renal tumour. Am J Med 1967;43:963-76.  Back to cited text no. 5
    
6.
Bedano PM, Bonnin J, Einhorn LH. Metachronous intracranial germinoma in a patient with a previous primary mediastinal seminoma. J Clin Oncol 2006;24:2386-7.  Back to cited text no. 6
    
7.
Ayyanar K, Blackman SC, Chordas C, Frazier L, Kieran MW. Metachronous mediastinal seminoma occurring after intracranial germinoma in an adolescent. J Pediatr Hematol Oncol 2009;31:861-4.  Back to cited text no. 7
    
8.
Matsui Y, Zsebo K, Hogan BL. Derivation of pluripotential embryonic stem cells from murine primordial germ cells in culture. Cell 1992;70:841-7.  Back to cited text no. 8
    
9.
Gitlin D, Perricelli A, Gitlin GM. Synthesis of fetoprotein by liver, yolk sac, and gastrointestinal tract of the human conceptus. Cancer Res 1972;32:979-82.  Back to cited text no. 9
    
10.
Murthy M, Xu S, Massimo G, Wolley M, Gordon RD, Stowasser M, et al. Role for germline mutations and a rare coding single nucleotide polymorphism within the KCNJ5 potassium channel in a large cohort of sporadic cases of primary aldosteronism. Hypertension 2014;63:783-9.  Back to cited text no. 10
    
11.
Mundy GR. Metastasis to bone: causes, consequences and therapeutic opportunities. Nat Rev Cancer 2002;2:584-93.  Back to cited text no. 11
    


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  [Figure 1], [Figure 2]



 

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