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| Year : 2021 | Volume
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| Issue : 1 | Page : 226-228 |
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| Catechism (Quiz 11) |
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Jayashri Chaudhari, Kanchan Kothari, Pradeep Vaideeswar
Department of Pathology, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India
Click here for correspondence address and email
| Date of Submission | 12-Jul-2020 |
| Date of Acceptance | 28-Jul-2020 |
| Date of Web Publication | 8-Jan-2021 |
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How to cite this article:
Chaudhari J, Kothari K, Vaideeswar P. Catechism (Quiz 11). Indian J Pathol Microbiol 2021;64:226-8 |
 Part 1 | |  |
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].
| Questions | | |
- What is the diagnosis?
- Which are the histological feature seen in the images?
- Has the excision being performed solely for cosmetic reasons?
Answers of Catechism (Quiz 10)
| Answers | | |
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 | | |
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 | | |
| 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. 5 th ed. Lyon, France: IARC Press; 2020. p. 150-1.  |
| 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. |
| 3. | Montgomery E, Epstein JI. Anastomosing hemangioma of the genitourinary tract: A lesion mimicking angiosarcoma. Am J Surg Pathol 2009;33:1364-9. |
| 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. |
| 5. | Bean GR, Joseph NM, Folpe AL, Horvai AE, Umetsu SE. Recurrent GNA14 mutations in anastomosing haemangiomas. Histopathology 2018;73:354-7. |
Correspondence Address:
Jayashri Chaudhari
Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai - 400 012, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/IJPM.IJPM_849_20
[Figure 1], [Figure 2] |
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