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LETTER TO THE EDITOR
Year : 2012  |  Volume : 8  |  Issue : 3  |  Page : 465-467

Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement


1 Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, India
2 Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India

Date of Web Publication17-Nov-2012

Correspondence Address:
Urmila Majhi
Department of Pathology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai - 600036
India
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DOI: 10.4103/0973-1482.103541

PMID: 23174743

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How to cite this article:
Majhi U, Murhekar K, Sundersingh S, Krishnamurthy A. Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. J Can Res Ther 2012;8:465-7

How to cite this URL:
Majhi U, Murhekar K, Sundersingh S, Krishnamurthy A. Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement. J Can Res Ther [serial online] 2012 [cited 2014 Dec 22];8:465-7. Available from: http://www.cancerjournal.net/text.asp?2012/8/3/465/103541

Sir,

Malignant ectomesenchymoma (MEM) is a rare tumor of soft tissues or the central nervous system, which is composed of both neuroectodermal elements and mesenchymal components. [1] We present here a case of malignant ectomesenchymoma involving paranasal sinuses with proptosis and nodal involvement.

A 40-year-old patient presented with pain and progressive diminution of vision and proptosis of right eye [Figure 1]a for last 6 months. On examination, the patient had multiple right cervical lymph nodes starting from level I to level V. Ear, nose and throat examination revealed a polypoidal mass in superior meatus. Biopsy of the nasal mass showed a malignant tumor composed of round, oval to slightly elongated cells with scanty cytoplasm and dark staining nuclei [Figure 1]b and [Figure 2]a. The cells were seen in sheets or clusters. The immunohistochemical studies revealed positive staining for vimentin, NSE, chromogranin [Figure 2]c, synaptophysin [Figure 2]d, CD-56 [Figure 3]a, CD-57 [Figure 3]b, desmin [Figure 3]d,and S-100p and negative reaction for LCA, keratin, CD 99, smooth muscle actin, melan A, HMB -45 and neurofilament. The Ki -67 index was 40% [Figure 3]c. Diagnosis of ectomesenchymoma was made due to the presence of both neuronal and rhabdomyomatous component.
Figure 1: (a) Picture showing right eye swelling., (b) H and E X 20-Respiratory mucosa of paranasal sinus partly replaced by tumor. The cells are of moderate size and round, oval to slightly elongated with scanty cytoplasm and hyperchromatic nuclei

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Figure 2: (a) HandE X 40 Round, oval to elongated tumor cells with hyperchromatic nuclei. A normal mucus gland is seen. (b) FNAC cervical lymph node X40 - Round, oval to elongated hyperchromatic tumor cells metastatic in lymph node. (c) IHC Chromogranin X 40- showing fi brillary staining. (d) IHC Synaptophysin X 40-showing fibrillary staining

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Figure 3: (a) IHC CD 56 X 40 showing strong membrane positivity. (b) IHC CD 57 X 40 s howing strong cytoplasmic positivity. (c) IHC Ki 67 X 40 around 40% cells strong nuclear positivity. (d) IHC Desmin X 40 many round oval to elongated tumor cells showing strong cytoplasmic staining

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Computerized tomography [Figure 4] revealed a large tumor involving right nasal cavity, maxillary, frontal and ethmoid sinuses extending into orbit with focal deficit in right side cribriform plate. Fine needle aspiration biopsy of the right cervical lymph node revealed metastatic malignant tumor [Figure 2]b. The tumor was very extensive and hence primary radical surgery was not possible. The patient was planned for high dose chemotherapy and radiotherapy and to be considered for surgery later.
Figure 4: CT scan showing tumor involving right paranasal sinuses

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Malignant ectomesenchymoma (MEM) is a rare fast growing tumor; occurs mainly in children, although cases have been reported in patients up to age 60 and involves soft tissues or the CNS, and is composed of both neuroectodermal elements represented by ganglion cells and/or well-differentiated or poorly differentiated neuroblastic cells such as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, peripheral primitive neuroectodermal tumors (PNET) and one or more mesenchymal neoplastic elements, usually rhabdomyosarcoma. [2],[3],[4] The most accepted theory suggests that this tumor arises from pluripotent primitive neural crest cells. Complete surgical resection is the mainstay of treatment. High-dose combination chemotherapy and radiation therapy also appear to be important especially when radical surgery is not possible. [1],[2],[3],[4]

 
 > References Top

1.Brehmer D, Overhoff HM, Marx A. Malignant ectomesenchymoma of the nose. Case report and review of the literature. ORL J Otorhinolaryngol Relat Spec 2003;65:52-6.  Back to cited text no. 1
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2.Oppenheimer O, Athanasian E, Meyers P, Antonescu CR, Gorlick R. Malignant ectomesenchymoma in the wrist of a Child: Case Report and Review of the Literature. Int J Surg Pathol 2005;13:113-6.  Back to cited text no. 2
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3.Kawamoto EH, Weidner N, Agostini RM Jr, Jaffe R. Malignant ectomesenchymoa of soft tissue report of two cases and review of the literature. Cancer 1987;59:1791-802.  Back to cited text no. 3
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4.Vinck AS, Lerut B, Hermans R, Nuyts S, Sciot R, Jorissen M. Malignant ectomesenchymoma of the paranasal sinuses with proptosis. B-ENT 2011;7:201-4.  Back to cited text no. 4
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