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Year : 2019  |  Volume : 62  |  Issue : 2  |  Page : 341-342
Mediastinal mixed germ cell tumor with splenic histiocytosis: A rare coincidence


1 Department of Pathology and Lab Services, Rajiv Gandhi Cancer Institute and Research Center, Rohini, Delhi, India
2 Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Center, Rohini, Delhi, India

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Date of Web Publication10-Apr-2019
 

How to cite this article:
Sood R, Mehta A, Bansal D, Singh N, Purohit SR. Mediastinal mixed germ cell tumor with splenic histiocytosis: A rare coincidence. Indian J Pathol Microbiol 2019;62:341-2

How to cite this URL:
Sood R, Mehta A, Bansal D, Singh N, Purohit SR. Mediastinal mixed germ cell tumor with splenic histiocytosis: A rare coincidence. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Jun 24];62:341-2. Available from: http://www.ijpmonline.org/text.asp?2019/62/2/341/255839




A 32-year-old male presented with acute left upper quadrant pain. He gave a clinical history of surgery performed 6 months back for nonseminomatous mediastinal germ cell tumor comprising of embryonal cell carcinoma and teratoma as major components (Slides reviewed: [Figure 1]a and [Figure 1]b). Contrast-enhanced Computed Tomography (CT)-scan abdomen showed splenomegaly with multiple ill-defined hypodense lesions within spleen. A clinical suspicion of splenic metastasis was raised and further investigations advised. Splenic Fine needle aspiration cytology (FNAC) was performed and cytology smears/cell block showed clusters of large pleomorphic cells with abundant eosinophilic cytoplasm [Figure 1]c,[Figure 1]d,[Figure 1]e. Immunohistochemical markers for germ cell tumors were all negative including SOX-2 and SALL4, hence ruling out the possibility of a splenic metastasis. Furthermore, a comprehensive panel of Immunohistochemistry (IHC) markers were applied including LCA, CK, desmin, synaptophysis, CD68, CD163, CD117, MPO, CD61, CD1a, CD21, and CD23 to identify the atypical cell lineage. All markers were negative except CD68, CD163 (weak and restricted) and S100 (focal) [Figure 1]f,[Figure 1]g,[Figure 1]h. Hence, a final diagnosis of mediastinal nonseminomatous germ cell tumor (NSGCT) with associated histiocytic sarcoma of non-Langerhans type was made.
Figure 1: (a) and (b) show embryonal carcinoma and teratoma components of germ cell tumor. Splenic aspirate (c) shows scattered large atypical cells with pleomorphic hyper chromatic nuclei. Cell block (d) and (e) is cellular and shows sheets of cells with similar morphology. On IHC, these cells express CD68 (strong) (f), CD163 (weak, restricted) (g) and are negative for SALL4 (h)

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Histiocytic proliferations associated with mediastinal nonseminomatous germ cell tumor (MNSGCT), ranging from hemophagocytic syndrome (HPS) to malignant histiocytosis (MH)/disseminated histiocytic sarcoma (HS), are very rare.[1] The prognosis of these patients is extremely poor, with survival measured in months. Standard treatment modalities are yet to be established. The use of thalidomide/ Cyclophosphamide, hydroxydaunorubicin, oncovin, prednisolone (CHOP) followed by alemtuzumab-containing reduced-intensity Peripheral blood stem cell transplantation (PBSCT) is ideal modality of treatment in such patients.[1],[2] Such lesions might be on the pathway of multistep tumorigenesis toward a final onset of acute leukemia.[3] Through this case report, we aim to create awareness about the rare association of the MNSGCT with histiocytic neoplasms in order to prevent overzealous diagnosis of splenic metastases without proper histopathological and IHC confirmation.

Disclosures

The identity of the patient is not disclosed here in this case.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Fang LH, Shih LS, Lee PI, Chen WT, Chen RL. Mediastinal germ cell tumor-associated histiocytic proliferations treated with thalidomide plus chemotherapy followed by alemtuzumab-containing reduced intensity allogeneic peripheral blood stem cell transplantation: A case report. Medicine (Baltimore) 2016;95:e2515.  Back to cited text no. 1
    
2.
Shinoda H, Yoshida A, Teruya-Feldstein J. Malignant histiocytoses/disseminated histiocytic sarcoma with hemophagocytic syndrome in a patient with mediastinal germ cell tumor. Appl Immunohistochem Mol Morphol 2009;17:338-44.  Back to cited text no. 2
    
3.
Song SY, Ko YH, Ahn G. Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association. Int J Surg Pathol 2005;13:299-303.  Back to cited text no. 3
    

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Correspondence Address:
Ridhi Sood
Department of Pathology and Lab Services, Rajiv Gandhi Cancer Institute and Research Centre, Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_691_18

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