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Year : 2021  |  Volume : 14  |  Issue : 1  |  Page : 60-63  

Extragonadal mixed germ cell tumor presenting as large mass in the pelvic cavity

1 Department of Pathology, Uttar Pradesh University of Medical Sciences, Etawah, Uttar Pradesh, India
2 Department of General Surgery, Uttar Pradesh University of Medical Sciences, Etawah, Uttar Pradesh, India

Date of Submission18-Aug-2019
Date of Decision06-Jan-2020
Date of Acceptance22-Nov-2020
Date of Web Publication22-Jan-2021

Correspondence Address:
Savita Agarwal
Department of Pathology, Uttar Pradesh University of Medical Sciences, Saifai, Etawah, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_237_19

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Extragonadal germ cell tumors are relatively rare tumor, which usually occurs in the mediastinum or retroperitoneum. In this report, we present a case of primary mixed germ cell tumor (GCT) arising in the pelvic cavity. A 38-year-old male with acute abdominal pain was admitted to our hospital. Computed tomography scan imaging demonstrated a large mass in the pelvic cavity. Histological examination of the specimen revealed germ cell tumor with dual morphology. Immunohistochemical studies detected placental alkaline phosphatase (ALP), OCT3/4, ALP, and pancytokeratin. Taking these results together with the patient's other clinical manifestations, this case was diagnosed with mixed GCT, i.e., seminoma plus yolk sac tumor. Chemotherapy was successful. Mixed germ cell tumor in the pelvic cavity is extremely rare but should be considered as a cause of pelvic mass formation.

Keywords: Alpha-fetoprotein, epithelial membrane antigen, immunohistochemistry, pancytokeratin, placental alkaline phosphatase

How to cite this article:
Aggarwal R, Pandey P, Agarwal S, Shukla V, Chaturvedi V, Singh SP. Extragonadal mixed germ cell tumor presenting as large mass in the pelvic cavity. Med J DY Patil Vidyapeeth 2021;14:60-3

How to cite this URL:
Aggarwal R, Pandey P, Agarwal S, Shukla V, Chaturvedi V, Singh SP. Extragonadal mixed germ cell tumor presenting as large mass in the pelvic cavity. Med J DY Patil Vidyapeeth [serial online] 2021 [cited 2021 Mar 9];14:60-3. Available from: https://www.mjdrdypv.org/text.asp?2021/14/1/60/307668

  Introduction Top

Primary germ cell tumors of the extragonadal origin are rare, and the exact incidence of this type of cancer is unknown. They have been estimated to represent 3%–5% of all adult germ cell malignancies.[1] The origin of primary extragonadal germ cell tumour (EGGCT) is still a matter of debate. According to widely accepted theory during embryogenesis, germ cells are misplaced anywhere in the midline. The mechanism of misplacement is poorly understood, but malignant transformation of these cells leads to EGGCT at these regions.[2],[3] The most common site of origin is in decreasing order of the mediastinum, retroperitoneum, sacrococcygeal region, and pineal gland; although many unusual sources have been reported.[4] We report a case of primary extragonadal germ cell tumor presenting as a pelvic mass.

  Case Report Top

A 38-year-old adult male was admitted to the Department of Surgery, Uttar Pradesh University of Medical Sciences, Saifai, Etawah, with the symptoms of abdominal pain. His history was unremarkable, and he had no urologic problems. No abnormality was observed during the physical and genital examination. The clinical diagnosis of acute appendicitis was made. The abdominal ultrasonography revealed 67 mm × 63 mm echogenic semisolid mass at the anterior right renal pelvic zone. The abdominal computed tomography (CT) showed large retroperitoneal tumor 5 cm × 4 cm × 4 cm in size. The tumor mass was localized between the right kidney and aorta. The fatty tissue between the mass and kidney were diminished. No echogenic pathology was obtained during scrotal ultrasonography. Extensive examination for metastasis, including chest X-ray, chest CT, and radionuclide bone scan demonstrated no abnormalities. The operation consisted of resection of tumor and the para-aortic lymph node dissection. Histopathological evaluation of the specimen revealed a germ cell tumor with dual morphology [Figure 1]a. The predominant component was that of yolk sac tumor showing reticular areas with  Schiller-Duval Bodies More Details [Figure 1]b. Along with this, there was sharply demarcated second tumor consistent with the morphology of seminoma consisting of uniform tumor cells with sharply outlined cell membranes and large central nuclei [Figure 1]c. The Periodic Acid-Schiff (PAS) staining revealed intracytoplasmic staining on PAS staining [Figure 1]d. Immunohistochemistry of the tumor was also performed, as shown in [Table 1].
Figure 1: (a) Germ cell tumor with sharply demarcated dual morphology (red arrow indicating yolk sac tumor and black arrow indicating seminoma) (H and E, ×40). (b) Glomeruloid pattern with Schiller-duval bodies representing yolk sac tumor (H and E, ×100). (c) Seminomatous area comprising of tumor cells arranged in nest having clear to vacuolated cytoplasm, large central nuclei with prominent nucleoli, and lymphocytes in the septa (H and E, ×400). (d) PAS-positive: tumor showing intracytoplasmic staining

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Table 1: Result of immunohistochemistry in the present case

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Immunohistochemical staining was strongly positive for alpha-fetoprotein (AFP) in the yolk sac tumor area [Figure 2] and placental alkaline phosphatase and OCT3/4 in seminoma area [Figure 3]a and [Figure 3]b. Para-aortic lymph nodes show features of reactive lymphoid hyperplasia with sinus histiocytosis. Final histopathological diagnosis was given as mixed germ cell tumor with components of yolk sac tumor and seminoma.
Figure 2: Immunohistochemical staining was strongly positive for alpha-fetoprotein in the yolk sac tumor area

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Figure 3: (a and b) Placental alkaline phosphatase and OCT3/4 in seminoma area

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Neoadjuvant chemotherapy using bleomycin, etoposide, and cisplatin (BEP) at an interval of 3 weeks was given. At the end of the third cycle, serum AFP levels dropped to normal. There was no evidence of recurrence after 1-year follow-up.

  Discussion Top

Primary germ cell tumors of extragonadal origin are rare. They have been estimated to represent 3%–5% of all adult germ cell malignancies.[1] EGGCTs are not clinically evident with a primary gonadal tumor, in virtually all patients, the diagnosis is led by the symptoms induced by the growing mediastinal or retroperitoneal tumor mass. The determination of serum markers and request for immunopathology with specific germ cell markers in the pathological specimen should result in the diagnosis of EGGCT in almost all cases.[5]

Differential diagnosis of pelvic mass, include primary tumors (bladder carcinoma, paraganglioma, liposarcoma, leiomyosarcoma, and malignant fibrous histiocytoma), inflammatory diseases (actinomycosis and pelvic tuberculosis), idiopathic retroperitoneal fibrosis, and lymphoproliferative disorders.[6] However, the incidence of mixed germ cell tumor presenting as pelvic mass has not been reported. In this case, the diagnosis was difficult before admission, as there were no symptoms during the early stage of the disease.

The cytogenetic finding of one or multiple copies of the short arm of chromosome 12p with the loss of the long arm of chromosome 12 is observed in nearly all germ cell cancers of primary gonadal and extragonadal origin.[7],[8]

EGGCTs occur in several characteristic histological patterns that reflect the stages of normal embryonic and fetal development with seminomatous and nonseminomatous germ cell tumors (GCTs), including endodermal sinus tumor, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mature or immature teratoma.[5]

In EGGCT, a treatment strategy similar to that for metastatic testicular germ cell tumor is indicated. For such cases, four cycles of BEP chemotherapy were recommended and surgical resection of residual tumor is also considered mandatory.[9],[10] In our case, we performed surgical resection after which the diagnosis of mixed germ cell tumor was given. On the postoperative 21st day, four courses of chemotherapy, consisting of BEP were administered every 3 weeks to the patient. Serum AFP levels have fallen to the normal range 18 weeks after the surgery. Despite the freedom from disease at 12-month follow-up, further careful observation is needed.

Primary mediastinal and retroperitoneal extragonadal seminomatous GCTs have an equivalent prognosis as their primary gonadal counterpart, with a 5-year survival of 88% Nonseminomatous EGGCTs have a worse prognosis than seminomatous EGGCTs with a 5-year survival rate of 62% for retroperitoneal and 45% for mediastinal nonseminomatous EGGCTs.[11] The outcome of patients relapsing after primary chemotherapy for EGGCTs is very poor and probably worse than patients with primary gonadal nonseminomatous testicular cancer and poor prognostic features.[12]

Burned-out testicular tumors can undergo spontaneous complete regression and may present as metastasis at different sites such as retroperitoneum, retroperitoneal lymph nodes, lungs, and liver.[13] Histologic features of regressed tumors are scarring, chronic lymphoplasmacytic infiltrate, intratubular calcifications, hemosiderin-containing histiocytes, along with testicular atrophy. In the present case, it was primary retroperitoneal GCT and features of tumor regression were not seen.[14]

In conclusion, primary mixed GCT in the pelvic cavity is extremely rare but should be considered for a differential diagnosis when an intrapelvic mass is detected, even in the absence of clinical symptoms because these tumors can be successfully treated by surgery and BEP chemotherapy like our patient with primary retroperitoneal mixed GCT (seminoma plus yolk sac tumor).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Garnick MB, Canellos GP, Richie JP. Treatment and surgical staging of testicular and primary extragonadal germ cell cancer. JAMA 1983;250:1733-41.  Back to cited text no. 1
Flanagan CW, Parker JR, Mannel RS, Min KW, Kida M. Primary endodermal sinus tumor of the vulva: A case report and review of the literature. Gynecol Oncol 1997;66:515-8.  Back to cited text no. 2
Park NH, Ryu SY, Park IA, Kang SB, Lee HP. Primary endodermal sinus tumor of the omentum. Gynecol Oncol 1999;72:427-30.  Back to cited text no. 3
Toner GC, Geller NL, Lin SY, Bosl GJ. Extragonadal and poor risk nonseminomatous germ cell tumors. Survival and prognostic features. Cancer 1991;67:2049-57.  Back to cited text no. 4
Bokemeyer C, Nichols CR, Droz JP, Schmoll HJ, Horwich A, Gerl A, et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: Results from an international analysis. J Clin Oncol 2002;20:1864-73.  Back to cited text no. 5
Menenakos C, Braumann C, Hartmann J, Jacobi CA. Retroperitoneal castleman's tumor and paraneoplastic pemphigus: Report of a case and review of the literature. World J Surg Oncol 2007;5:45.  Back to cited text no. 6
Samaniego F, Rodriguez E, Houldsworth J, Murty VV, Ladanyi M, Lele KP, et al. Cytogenetic and molecular analysis of human male germ cell tumors: chromosome 12 abnormalities and gene amplification. Genes chromosomes cancer.1990;1:289-300.  Back to cited text no. 7
Chaganti RS, Houldsworth J. Genetics and biology of adult human male germ cell tumors. Cancer Res 2000;60:1475-82.  Back to cited text no. 8
Schmoll HJ. Extragonadal germ cell tumors. Ann Oncol 2002;13 Suppl 4:265-72.  Back to cited text no. 9
Andac A, Mert B, Sevil B, Burak S, Haluk O. Adult primary extragonadal germ cell tumors: Treatment results and long-term follow-up. Med Pediatr Oncol 2003;41:49-53.  Back to cited text no. 10
Bokemeyer C, Droz JP, Horwich A, Gerl A, Fossa SD, Beyer J, et al. Extragonadal seminoma: An international multicenter analysis of prognostic factors and long term treatment outcome. Cancer 2001;91:1394-401.  Back to cited text no. 11
Hartmann JT, Einhorn L, Nichols CR, Droz JP, Horwich A, Gerl A, et al. Second-line chemotherapy in patients with relapsed extragonadal nonseminomatous germ cell tumors: Results of an international multicenter analysis. J Clin Oncol 2001;19:1641-8.  Back to cited text no. 12
Budak S, Celik O, Turk H, Suelozgen T, Ilbey YO. Extragonadal germ cell tumor with the “burned-out” phenomenon presented a multiple retroperitoneal masses: A case report. Asian J Androl 2015;17:163-4.  Back to cited text no. 13
[PUBMED]  [Full text]  
Balalaa N, Selman M, Hassen W. Burned-out testicular tumor: A case report. Case Rep Oncol 2011;4:12-5.  Back to cited text no. 14


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