Journal of Cancer Research and Therapeutics

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

Malignant ectomesenchymoma of paranasal sinuses with proptosis and nodal involvement


Urmila Majhi1, Kanchan Murhekar1, Shirley Sundersingh1, Arvind Krishnamurthy2,  
1 Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, India
2 Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India

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




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;8:465-467


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 2022 Jan 21 ];8:465-467
Available from: https://www.cancerjournal.net/text.asp?2012/8/3/465/103541


Full Text

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}{Figure 2}{Figure 3}

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}

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

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2Oppenheimer 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.
3Kawamoto 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.
4Vinck 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.