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QUIZ  
Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 226-228
Catechism (Quiz 11)


Department of Pathology, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India

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Date of Submission12-Jul-2020
Date of Acceptance28-Jul-2020
Date of Web Publication8-Jan-2021
 

How to cite this article:
Chaudhari J, Kothari K, Vaideeswar P. Catechism (Quiz 11). Indian J Pathol Microbiol 2021;64:226-8

How to cite this URL:
Chaudhari J, Kothari K, Vaideeswar P. Catechism (Quiz 11). Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Jan 16];64:226-8. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/226/306539





   Part 1 Top


Catechism

A 16-year-old girl complained of thickening of skin on the right cheek, which had been present for the past 5 years. The lesion was excised. A 3.5 × 1.5 × 0.2 cm skin-covered tissue was received in the surgical pathology laboratory. It showed a flesh colored, plaque-like, hairless lesion with rough surface. The histopathological features are seen in [Figure 1].
Figure 1:

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   Questions Top


  1. What is the diagnosis?
  2. Which are the histological feature seen in the images?
  3. Has the excision being performed solely for cosmetic reasons?


Answers of Catechism (Quiz 10)


   Answers Top


Microscopic examination revealed a vascular neoplasm, focally infiltrating into the adjacent tissues and composed of anastomosing sinusoidal capillary sized blood vessels with hobnail endothelial cells. There were no mitotic figures or cellular multilayering. There were areas of sclerosis. However, there were no areas of tumor diathesis [Figure 1] and [Figure 2]. Immunohistochemically, the tumor cells were positive for CD34, CD31 and Fli1 [Figure 3].
Figure 3: (a) CD34 highlighting the tumor. Diaminobenzidine, ×200. (b) Diffuse Fli1 positivity. DAB, ×400

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Answer1: Anastomosing Hemangioma.

Answer 2: CD34, CD31, Fli1, ERG.

Answer 3: Inactivating hot spot mutation in GNAQ or GNA14.


   Discussion Top


Anastomosing hemangioma is included in the recent, 5th edition of the World Health Organization (WHO) fascicle of soft tissue and bone tumors and is defined as a benign vascular neoplasm, consisting of thin-walled anastomosing blood vessels, lined by a monolayer of plump, protruding endothelial cells.[1]

Although this tumor was initially reported in the male genital tract, its most common site is kidney and retroperitoneal adipose tissue, as noted in the present case. Over a period of time, this tumor has been reported in other sites, including liver, paravertebral soft tissues, mediastinum, etc.[2]

Microscopically, this tumor is infiltrative and exhibits a nonlobular or a loosely lobular architecture, including sinusoidal capillary-sized vessels with mild endothelial cell nuclear variation and scattered endothelial cells with hobnail nuclei, as noted in the present case. Mitotic activity is rare or absent, as similarly observed in this case. Adipocytic metaplasia and extramedullary hematopoiesis are noted in subsets of cases.[1],[2],[3] These lesions can be diagnostically difficult and misdiagnosed as an angiosarcoma, especially on a limited biopsy sample.

Immunohistochemically, the tumor cells show positive expression of endothelial markers, such as CD34, CD31, Fli1, and ERG.[1],[2],[3] Lately, inactivating hot spot mutation in GNAQ or GNA14 has been reported in a vast majority of anastomosing hemangiomas.[4],[5]

Diagnosis of “atypical cells” on the initial biopsy, in the present case, indicates an intrinsic challenge associated while recognizing this entity, as similarly noted earlier, especially in cases occurring at unconventional sites.[2]Therefore, awareness of this tumor entity and correlation of microscopic features with radiologic imaging, especially for tumor location, constitute as useful clues for its correct and timely diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.









 
   References Top

1.
Montgomery EA, Umetsu SE. Vascular tumors/Anastomosing hemangioma. In: World Health Organization (WHO) classification of tumours editorial board, editors. World Health Organization Classification of Tumours. Soft Tissue and Bone Tumours. 5th ed. Lyon, France: IARC Press; 2020. p. 150-1.  Back to cited text no. 1
    
2.
John I, Folpe AL. Anastomosing hemangiomas arising in unusual locations: A clinicopathologic study of 17 soft tissue cases showing a predilection for the paraspinal region. Am J Surg Pathol 2016;40:1084-9.  Back to cited text no. 2
    
3.
Montgomery E, Epstein JI. Anastomosing hemangioma of the genitourinary tract: A lesion mimicking angiosarcoma. Am J Surg Pathol 2009;33:1364-9.  Back to cited text no. 3
    
4.
Bean GR, Joseph NM, Gill RM, Folpe AL, Horvai AE, Umetsu SE. Recurrent GNAQ mutations in anastomosing hemangiomas. Mod Pathol 2017;30:722-7.  Back to cited text no. 4
    
5.
Bean GR, Joseph NM, Folpe AL, Horvai AE, Umetsu SE. Recurrent GNA14 mutations in anastomosing haemangiomas. Histopathology 2018;73:354-7.Back to cited text no. 5
    

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Correspondence Address:
Jayashri Chaudhari
Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_849_20

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    Figures

  [Figure 1], [Figure 2]



 

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