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Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 444-445
Presacral medulloepithelioma

1 Department of Pathology, PSG Institute of Medical Science and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu, India
2 Department of Paediatric Surgery, PSG Institute of Medical Science and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu, India

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How to cite this article:
Pillai SB, Kumar R K, Ganesan PA, Nirmala V. Presacral medulloepithelioma. Indian J Pathol Microbiol 2008;51:444-5

How to cite this URL:
Pillai SB, Kumar R K, Ganesan PA, Nirmala V. Presacral medulloepithelioma. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Jul 3];51:444-5. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/444/42519


Medulloepithelioma is a rare malignant embryonal tumor affecting young children between 6 months and 5 years of age. The most common site is periventricular within the cerebral hemispheres. [1]

A 3-year-old girl presented with swelling below the coccyx of 7 months' duration. The swelling was slowly increasing in size. She was an active afebrile child with a 4 5 cm swelling in the natal cleft extending onto the right gluteal region. It was cystic in consistency and non-transluminant. The spine was normal. Right inguinal lymph node measuring 2cm was palpable. No other swelling elsewhere was detected.

Hematological, biochemical and serological investigations were within normal limits. Serum alpha-fetoprotein was 0.984 IU/ml (Normal - <11.3 IU/ml). Ultrasound abdomen showed bilateral iliac lymphadenopathy, more on the right side.

Magnetic resonance imaging revealed a large heterogeneously enhancing mass lesion in the right gluteal region with perineal extension and multiple hyperintense signals along the left and right iliac vessels due to, probably, enlarged lymph nodes. X-ray chest was normal.

Fine needle aspiration cytology of the swelling was done, which showed highly cellular smears with clusters of small round cells having high nuclear cytoplasmic ratio and finely reticulated nuclear chromatin. Diagnosis of small round cell tumor was made.

A provisional clinical diagnosis of sacro-coccygeal teratoma was entertained and excision of the mass and coccyx along with biopsy of the right inguinal lymph node and bone marrow were done.

The excised mass was globular and measured 6.0 4.5 3.0 cm. External surface was rough with adipose tissue at one end. Cut surface showed friable polypoidal projections into a cystic space measuring 5.0 cm across. The cystic portion had a smooth lining. Adjoining this was a solid area with foci of hemorrhage.

Histopathological examination showed a partly cystic neoplasm with a thin fibrous capsule and the cyst was lined by immature neuroepithelial cells. The solid areas consisted of mitotically active immature neuroepithelium with hyperchromatic nuclei arranged in tubules [Figure 1], rosettes, trabeculae and papillae, with an external limiting membrane [Figure 2].

Foci of delicate mesenchymal stroma were present in between the tumor cells. Also seen were areas of hyalinization, necrosis and focal calcification. Endodermal and mesodermal derivatives were not detected. A histopathological diagnosis of medulloepithelioma was made. Immunohistochemical stains demonstrated diffuse basal positivity for vimentin. [Figure 3] Coccyx and the lymph node also showed tumor deposits with similar morphology. Bone marrow was free of tumor.

Medulloepithelioma is a rare tumor of embryonal neuroepithelial origin. Less than 40 cases have been reported. So far, only seven cases of peripheral medulloepitheliomas have been reported, with sites including presacral region, pelvis and along the sciatic nerve. [2]

Histologically, medulloepitheliomas resemble the embryonic neural tube, characterized by papillary, tubular, or trabecular arrangement of neoplastic neuroepithelium with an external limiting membrane. Multiple lines of differentiation including neural, glial and mesenchymal elements may be displayed. [1]

In the present case, the main differential diagnosis is the more common immature teratoma, due to its location. Sacrococcygeal teratomas are composed of a wide diversity of tissues foreign to the anatomic site in which they arise and are thought to originate from totipotent cells. Histologically, they are characterized by the presence of immature elements from all the three germ cell layers.

In our case, even with extensive sampling, the tumor showed only structures resembling the primitive neural tube and elements representing other germ cell layers were absent. Teratomas can be monodermal, where one component has overgrown the teratoma in which it originated. In most monodermal teratomas, other elements are discernible at least in small foci. However, pure monodermal teratomas, though extremely rare, do occur.

Presacral medulloepitheliomas arise from undifferentiated embryonic cells forming the presacral remnants of the neurenteric canal. Since both medulloepithelioma and teratoma are embryonal multipotential tumors, peripheral medulloepitheliomas may represent the neural component of immature teratoma and can be classified under pure monodermal teratoma. [3]

Medulloepithelioma cells express nestin, vimentin and microtubule-associated protein 5 similar to the immunoreactivity of cells of primitive neural tube. These cells do not express glial fibrillary acidic protein, neuron-specific enolase, or S100.

Due to the rarity of this neoplasm, optimal management is not known. Total surgical resection followed by radiotherapy is often the mode of treatment. Central medulloepitheliomas are highly malignant and have poor prognosis. Most children die within a year of diagnosis. There are reports of long-term survivors after total resection. [4],[5] Seven cases of peripheral medulloepithelioma have been reported, of which four cases were presacral medulloepithelioma. [2],[3],[5] All the seven cases of peripheral medulloepithelioma were primarily treated by excision. Our case had total resection of the tumor and coccyx with right inguinal lymph node biopsy followed by adjuvant chemotherapy and radiotherapy. After 5 months of follow-up, there is no evidence of local recurrence.

Presacral medulloepithelioma is an extremely rare neoplasm. Correct diagnosis and proper classification of this tumor are necessary to predict the clinical behavior and for optimal management.

   Acknowledgments Top

We thank Dr. Prasanna N. Kumar, Professor and Head, Department of Pathology and Dr. Vanitha S, Associate Professor for their outstanding support.

   References Top

1.Lopes MB, Vandenberg SR. Tumors of the central nervous system. In : Christopher D, Fletcher M, editors. Diagnostic histopathology of tumors. 2 nd ed. Philadelphia: Churchill Livingstone; 2003. p. 1650.  Back to cited text no. 1    
2.Somjee S, Craver R, Kallagowdar C. Presacral medulloepithelioma: De novo or teratomatous. J Pediatr Hematol Oncol 2004;21:85-91.  Back to cited text no. 2    
3.Donner LR, Teshima I. Peripheral medulloepithelioma: An immunohistochemical, ultrastructural and cytogenetic study of a rare, chemotherapy-sensitive pediatric tumor. Am J Surg Pathol 2003;27:1008-12.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Vincent S, Dhellemmes P, Maurage CA. Intracerebral medulloepithelioma with a long survival. Clin Neuropathol 2002;21:197-205.  Back to cited text no. 4    
5.Takei H, Florez L, Moroz K. Medulloepithelioma: Two unusual locations. Pathol Int 2007;57:91-5.  Back to cited text no. 5    

Correspondence Address:
Suma Bhargavan Pillai
Department of Pathology, PSG Institute of Medical Science and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.42519

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  [Figure 1], [Figure 2], [Figure 3]

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