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 2017-05-25  Case of the week # 447  © Andrei Telitchenko  www.TheFetus.net
Answer to the case of the week # 447
June 8, 2017 - June 22, 2017 

Andrew Averyanov, MD ; Andrei Telitchenko, MD.
Ukraine


Case report

A 24-year old (G1P0) woman with non-contributive history was referred to our unit at 38 weeks of gestation to evaluate a tumor located on the face. 
Our ultrasonographic examination revealed 32mm x 30mm x 30 mm tumor arising from right choana. 
It appeared as an echogenic soft tissue mass with cystic component. Color Doppler revealed vascularization mainly in the interior of the tumor. It was possible to follow the vessels into the nose. No bone defect nor anomalies in the brain structure were detected. 
Based on these ultrasound findings, hemangioma was suspected.

An elective caesarean section was performed at 39 weeks of gestation to avoid fetal trauma during vaginal delivery. 

A 3400 gr male baby was delivered with Apgar scores of 7 and 8. 
CT found no abnormalities of the skull or brain. 
A complete surgical excision of the tumor and plastic correction of the nose was done at 11
th day after delivery. Baby was doing well without complications.

The histological diagnosis was glomangioma.

glomus tumor (also known as a "solitary glomus tumor", "solid glomus tumor, or "glomangioma [1]) is a rare neoplasm arising from the glomus body. They account for less than 2% of all soft tissue tumors [2]. The majority of glomus tumors are benign, but they can also show malignant features [3]. Histologically, glomus tumors are made up of an afferent arteriole, anastomotic vessel, and collecting venule. Familial glomangiomas have been associated with a variety of deletions in the GLMN (glomulin) gene, and are inherited in an autosomal dominant manner, with incomplete penetrance [4].
Glomus tumors are usually solitary and small lesions. The vast majority are found in the distal extremities, particularly in the hand, wrist, foot, and under the fingernails [2]
 

 

Image 1 and video 1: Tumor arising from the nose channel and fetal naris.

 



Images 2-4, videos 2-3: 2D-3D views showing the tumor near the nose and its source in nasal channel.

  



Image 5, Videos 4 - 6: Doppler spectrum images showing that vascularization in the tumor tissues.





Image 6: Doppler image shows normal brain structure.


 



References
  1. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. 
  2. International Agency for Research on Cancer (2002). Pathology and Genetics of Tumours of Soft Tissue and Bone. St. Louis: WHO Press. pp. 136–137.
  3. De Chiara, Annarosaria; Apice, Gaetano; Mori, Stefano; Silvestro, Giustino; Losito, Simona N.; Botti, Gerardo; Ninfo, Vito (2003). "Malignant Glomus Tumour: A Case Report and Review of the Literature". Sarcoma. 7 (2): 87–91.w
  4. Gombos, Z; Zhang, PJ (September 2008). "Glomus tumor". Archives of pathology & laboratory medicine. 132 (9): 1448–52.
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