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Year : 2010  |  Volume : 53  |  Issue : 3  |  Page : 592-593
Extragonadal yolk sac tumor of face in a female infant: A case report

1 Department of Pathology, Gujarat Cancer & Research Institute (M.P. Shah Cancer Hospital), NCH Campus, Ahmedabad, Gujarat, India
2 Department of Radiodiagnosis, Gujarat Cancer & Research Institute (M.P. Shah Cancer Hospital), NCH Campus, Ahmedabad, Gujarat, India

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Date of Web Publication22-Oct-2010

How to cite this article:
Pasricha S, Gupta A, Shah M, Vadodaria H. Extragonadal yolk sac tumor of face in a female infant: A case report. Indian J Pathol Microbiol 2010;53:592-3

How to cite this URL:
Pasricha S, Gupta A, Shah M, Vadodaria H. Extragonadal yolk sac tumor of face in a female infant: A case report. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Dec 9];53:592-3. Available from: https://www.ijpmonline.org/text.asp?2010/53/3/592/68269


Yolk sac tumors (YSTs) are neoplasms of germ cell origin of gonadal sites. Extragonadal YST are uncommon and are particularly seen in sacrococcygeal, mediastinal, intracranial and retroperitoneal sites. [1] Extracranial head and neck region is an exceedingly rare site for YST and very few reports are documented over the lateral side of face as its primary site. [2],[3] We present a case of primary extragonadal YST involving right facial region.

A nine-month-old female presented with right-sided facial swelling for 2 to 3 months. Local examination revealed a 4 Χ 5 cm non-tender, firm, non-mobile mass in the right lateral side of the face. Routine laboratory investigations were normal. Magnetic resonance imaging (MRI) revealed a 32 Χ 30 Χ 56 mm well-defined mass in the right masticator space [Figure 1]a. An incisional biopsy was performed. Microscopically [Figure 2], the tumor showed network of microcystic spaces lined by discohesive large round to polygonal cells with vesicular, hyperchromatic nuclei, distinct central nucleoli and vacuolated cytoplasm. The tumor cells were intricately associated with loose vascular myxoid stroma. Few hyaline globules were identified that were periodic acid-Schiff-positive and diastase-resistant. Hemorrhage, necrosis and significant mitoses were present. No teratomatous component was identified. Immunohistochemistry (IHC) results showed positivity for cytokeratin (CK) and alfa-fetoprotein (AFP) [Figure 3]. An elevated serum AFP level of 18 964 ng/ml (normal range: 1-15) supported the diagnosis. To rule out the metastatic origin, ultrasonography (USG) of abdomen and pelvis was done, which was unremarkable with normal ovaries. Bone marrow examination, cerebrospinal fluid examination and bone scan were also normal. Above findings led to four cycles of multiagent chemotherapy (cisplatin, etoposide and bleomycin), which led to significant regression of mass, however, with a residual lesion in the masticator space which could be fibrosis or residual tumor [Figure 1]b and return of serum AFP to a normal value. Patient was lost to follow-up. Devaney and Ferlito [3] found 27 cases of YST of head and neck region. In one-third of the cases, YST was associated with teratoma and the rest with malignant GCT or non-germ cell malignancies. In the present case, no other tumor component was identified. The following differential diagnoses were considered: 1) germ cell tumor; 2) rhabdomyosarcoma; 3) liposarcoma and 4) undifferentiated carcinoma. Morphologically, microcystic pattern, discohesive cells, with vacuolated cytoplasm, spindling of tumor cells in myxoid stroma and hyaline globules favored the diagnosis of YST. IHC results along with markedly elevated serum AFP levels confirmed the diagnosis. Yolk sac tumors were fatal in the past. However, in the last decade, adjunct chemotherapy after surgery has produced a significant improvement in the survival rate. More recently, a regimen of bleomycin, etoposide and cisplatin (BEP) has been a major advance in the therapy of advanced and localized GCT. [4] Our patient underwent four cycles of multiagent chemotherapy (BEP) and has shown a dramatic response.
Figure 1: (a) MRI showing hyperintense mass lesion involving the right masti cator space, (b) Post-chemotherapy MRI showing signifi cant reducti on in size of the mass

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Figure 2: (a) YST showing microcysti c spaces lined by round to cuboidal tumor cells (H and E, ×100), (b) Round to polygonal pleomorphic tumor cells with vesicular chromati n and conspicuous nucleoli, Few hyaline globules are seen in the centre (H and E, ×400)

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Figure 3: (a) Tumor cells showing strong positi vity for CK (IHC stain, ×400), (b) Tumor cells showing positi vity for AFP (IHC stain, ×400)

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Awareness of the clinicians and pathologists for extragonadal YST at such unusual sites together with early recognition and the use of more efficacious combination chemotherapy will lead to a better therapeutic results and improved prognosis.

   References Top

1.Kusumakumari P, Geetha N, Chellam VG, Nair MK. Endodermal sinus tumors in head and neck region. Med Pediatr Oncol 1997;29:303-7.  Back to cited text no. 1  [PUBMED]    
2.Kebudi R, Ayan I, Darendeliler E, Agaoglu L, Kinay M, Olgac V, et al. Non-midline endodermal sinus tumor in the head and neck region: A case report. Med Pediatr Oncol 1993;21:685-9.  Back to cited text no. 2      
3.Devaney KO, Ferlito A. Yolk sac tumors (endodermal sinus tumors) of extracranial head and neck regions. Ann Otol Rhinol Laryngol 1997;106:254-60.  Back to cited text no. 3  [PUBMED]    
4.Dede M, Pabuccu R, Yagci G, Yenen MC, Goktolga U, Gunhan O. Extragonadal yolk sac tumor in pelvic localization: A case report and review of literature. Gynecol Oncol 2004;92:989-91.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  

Correspondence Address:
Sunil Pasricha
Department of Pathology, The Gujarat Cancer & Research Institute, (M.P. Shah Cancer Hospital), NCH Campus, Asarwa, Ahmedabad - 380 016, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.68269

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  [Figure 1], [Figure 2], [Figure 3]

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