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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 34-36

Clinical presentation of advanced extragonadal embryonal carcinoma mimicking classical hodgkin lymphoma


Division of Hematology/Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan

Date of Submission19-Apr-2021
Date of Decision15-Jun-2021
Date of Acceptance05-Jul-2021
Date of Web Publication07-Mar-2022

Correspondence Address:
Dr. Tsung-Ying Yu
Division of Hematology/Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu District, Taipei, 11490
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JCRP.JCRP_17_21

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  Abstract 


A 22-year-old man presented with a 2-week history of abdominal pain, fatigue, fever, and night sweats. Enlarged neck and paraaortic lymph nodes were noted, but no testicular lesions were found using imaging tools. Pathology revealed poorly differentiated embryonal carcinoma (EC) of the metastatic neck lymph node. The patient presented with clinical symptoms similar to Hodgkin lymphoma (HL), but the final diagnosis was advanced-stage extragonadal EC. To our knowledge, this is a rare case report of EC presenting as classic HL.

Keywords: Embryonal carcinoma, extragonadal, Hodgkin lymphoma


How to cite this article:
Liu WN, Yu TY. Clinical presentation of advanced extragonadal embryonal carcinoma mimicking classical hodgkin lymphoma. J Cancer Res Pract 2022;9:34-6

How to cite this URL:
Liu WN, Yu TY. Clinical presentation of advanced extragonadal embryonal carcinoma mimicking classical hodgkin lymphoma. J Cancer Res Pract [serial online] 2022 [cited 2022 Jul 1];9:34-6. Available from: https://www.ejcrp.org/text.asp?2022/9/1/34/339173




  Introduction Top


In general, embryonal carcinoma (EC) occurs at a young age, in the second to third decades on average.[1] EC is aggressive and results in early hematogenous spread. Approximately 66% of patients with EC have metastasis at the time of diagnosis.[2] It can be difficult to differentiate from lymphoma, with both diseases presenting with lymphadenopathy. Early histological proof and quick treatment are important factors for disease outcomes.


  Case Report Top


We present the case of a 22-year-old male patient who was admitted to our hospital with abdominal pain and fatigue. He had no underlying diseases or operation history. A clinical examination revealed a left supraclavicular fossa mass without painful sensation. No aberrant palpable masses over the scrotum or the inguinal canal were noted. Blood test results were normal (white blood cell count: 9.8 × 109/l, Hgb: 13.7 g/dl, blood creatinine: 0.8 mg/dl, C-reactive protein: 0.9 mg/dl). However, serum tumor markers were elevated: alpha-fetoprotein (αFP) (26 ng/ml), free human chorionic gonadotrophin (hCG) (0.17 ng/ml), lactate dehydrogenase (LDH) (485 U/l).

Abdominal contrast-enhanced computed tomography (CECT) showed weakly enhancing soft-tissue lesions in the paraaortic space [Figure 1]. Splenomegaly was also noted. Neck CECT showed a mass lesion in Level III and also in some small lymph nodes in the bilateral neck [Figure 2]. Scrotal sonography showed no significant varicocele or abnormal findings. Positron emission tomography (PET) showed an enhancing signal over the right subclavicular node and a paraaortic mass lesion [Figure 3]a.
Figure 1: Abdominal contrast-enhanced computed tomography showed weakly enhancing soft tissue lesions in the paraaortic space (white arrow)

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Figure 2: Neck contrast-enhanced computed tomography showed a mass lesion over the left neck (white arrow)

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Figure 3: (a) Positron emission tomography scan before treatment showed an enhancing signal over the right subclavicular node and paraaortic mass lesion (black arrow). (b) Positron emission tomography scan after four courses of bleomycin, etoposide, and cisplatin showed a residual enhancing lesion over the paraaortic region (black arrow)

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An excisional biopsy of the neck mass was done, and a histopathological examination showed poorly differentiated carcinoma. Immunohistochemistry staining was positive for cytokeratin (CK), CK7, CD30, sal-like protein 4, and octamer-binding transcription factor 3/4 (OCT 3/4) [Figure 4]. Metastatic EC of the neck mass was diagnosed. According to the American Joint Committee on Cancer staging system, cT0pN2M1aS2, Stage IIIB was diagnosed.
Figure 4: Immunohistochemistry staining. (a) H and E stain, (b) CD30, (c) Sal-like protein 4, (d) Octamer-binding transcription factor 4

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Following the NCCN guidelines, systemic chemotherapy with bleomycin, etoposide, and cisplatin was carried out at full dose for four courses. A follow-up PET showed a partial response [Figure 3]b. Further surgical interventions were needed, but the patient has so far refused.


  Discussion Top


EC is the second most common histological type of testicular tumor after seminoma.[3] In general, it occurs at a young age.[1] Germ cell tumors (GCTs) originate in the gonads in most clinical cases. Extragonadal germ cell tumors (EGGCTs) commonly arise in the midline of the retroperitoneum or the mediastinum but are not common.[4] Only approximately 2%–5% of all GCTs are of extragonadal origin.[5] The overall incidence ranges from 1.8 to 3.4/1 million.[6] According to this low incidence and nonspecific clinical features, diagnosing EC is a challenge, especially to differentiate it from Hodgkin lymphoma (HL).

The clinical presentations of EGGCTs depend on the location and size of the tumor. The symptoms of mediastinal GCTs include dyspnea, chest pain, fever, cough, and hemoptysis. In retroperitoneal GCTs, abdominal pain, lumbago, backache, and body weight loss are usually noted.[7]

Clinical symptoms and signs of HL in young adolescents include lymphadenopathy, systemic complaints, and mediastinal mass.[8] Patients may present with symptoms including fatigue, anorexia, and weight loss. Other symptoms such as fever and night sweats are also common.[9] Both EC and HL have similar clinical presentations, however, they differ in primary location (i.e. the testis for EC but not for HL). It is difficult to differentiate quickly when extragonadal EC occurs.

Serum tumor markers include αFP, βhCG, and LDH, which are a common tool for diagnosing EGGCTs. In EC, serum tumor markers including αFP, β-hCG, and LDH may be only slightly elevated and are thus hard to detect initially.[10]

As CD30 immunostaining is often observed in EC and also in a variety of lymphomas (e.g. HL, diffuse large B-cell lymphoma, and anaplastic large-cell lymphoma), CD30 should not be used as a marker to distinguish between EC and lymphomas.[11] The HL H and E stain may mimic EC closely because of its sheet-like growth pattern. In contrast to HL, CK immunostaining should highlight EC, and OCT4 may be used as a marker of either EC or seminoma.[12]

An early confirmatory diagnosis of EC is advantageous because of the high risk of lymphatic and vascular invasion, as well as the increased possibility of spread into the paratesticular tissue in EC.[13]

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Krag Jacobsen G, Barlebo H, Olsen J, Schultz HP, Starklint H, Søgaard H, et al. Testicular germ cell tumours in Denmark 1976-1980. Pathology of 1058 consecutive cases. Acta Radiol Oncol 1984;23:239-47.  Back to cited text no. 1
    
2.
Rodriguez PN, Hafez GR, Messing EM. Nonseminomatous germ cell tumor of the testicle: Does extensive staging of the primary tumor predict the likelihood of metastatic disease? J Urol 1986;136:604-8.  Back to cited text no. 2
    
3.
Kao CS, Ulbright TM, Young RH, Idrees MT. Testicular embryonal carcinoma: A morphologic study of 180 cases highlighting unusual and unemphasized aspects. Am J Surg Pathol 2014;38:689-97.  Back to cited text no. 3
    
4.
Khan L, Verma S, Singh P, Agarwal A. Testicular embryonal carcinoma presenting as chest wall subcutaneous mass. J Cytol 2009;26:39-40.  Back to cited text no. 4
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5.
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
    
6.
Stang A, Trabert B, Wentzensen N, Cook MB, Rusner C, Oosterhuis JW, et al. Gonadal and extragonadal germ cell tumours in the United States, 1973-2007. Int J Androl 2012;35:616-25.  Back to cited text no. 6
    
7.
Gao Y, Jiang J, Liu Q. Extragonadal malignant germ cell tumors: A clinicopathological and immunohistochemical analysis of 48 cases at a single Chinese institution. Int J Clin Exp Pathol 2015;8:5650-7.  Back to cited text no. 7
    
8.
Nachman JB, Sposto R, Herzog P, Gilchrist GS, Wolden SL, Thomson J, et al. Randomized comparison of low-dose involved-field radiotherapy and no radiotherapy for children with Hodgkin's disease who achieve a complete response to chemotherapy. J Clin Oncol 2002;20:3765-71.  Back to cited text no. 8
    
9.
Gobbi PG, Cavalli C, Gendarini A, Crema A, Ricevuti G, Federico M, et al. Reevaluation of prognostic significance of symptoms in Hodgkin's disease. Cancer 1985;56:2874-80.  Back to cited text no. 9
    
10.
Gilligan TD, Seidenfeld J, Basch EM, Einhorn LH, Fancher T, Smith DC, et al. American Society of Clinical Oncology Clinical Practice Guideline on uses of serum tumor markers in adult males with germ cell tumors. J Clin Oncol 2010;28:3388-404.  Back to cited text no. 10
    
11.
Higgins JP, Warnke RA. CD30 expression is common in mediastinal large B-cell lymphoma. Am J Clin Pathol 1999;112:241-7.  Back to cited text no. 11
    
12.
McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: A review with emphasis on pathologic features, clinical prognostic variables, and differential diagnostic considerations. Adv Anat Pathol 2007;14:69-92.  Back to cited text no. 12
    
13.
Moul JW, McCarthy WF, Fernandez EB, Sesterhenn IA. Percentage of embryonal carcinoma and of vascular invasion predicts pathological stage in clinical stage I nonseminomatous testicular cancer. Cancer Res 1994;54:362-4.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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