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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 160
Glomeruloid hemangioma in normal individuals

1 Department of Dermatology and STD, National Institute of Pathology, Indian Council of Medical Research, Safdarjung Hospital, New Delhi, India
2 Health Research, National Institute of Pathology, Indian Council of Medical Research, Safdarjung Hospital, New Delhi, India

Date of Web Publication5-Mar-2013

Correspondence Address:
Jyoti Gupta
Department of Dermatology and STD, Vardhaman Mahavir Medical College and Safdarjung Hospital, w 5, Green Park, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.108088

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Glomeruloid hemangioma is a rare, histologically distinctive, cutaneous, benign vascular tumor, originally described by Chan et al. Glomeruloid hemangioma appears specific to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (POEMS), and is usually, but not always, related to Castleman's disease. We herein report two cases with glomeruloid hemangioma, without any features of the POEMS syndrome. Glomeruloid hemangioma requires a high index of suspicion for diagnosis. It could rarely present as a solitary finding, without any evidence of an underlying POEMS syndrome.

Keywords: Castleman′s disease, glomeruloid hemangioma, POEMS syndrome

How to cite this article:
Gupta J, Kandhari R, Ramesh V, Singh A. Glomeruloid hemangioma in normal individuals. Indian J Dermatol 2013;58:160

How to cite this URL:
Gupta J, Kandhari R, Ramesh V, Singh A. Glomeruloid hemangioma in normal individuals. Indian J Dermatol [serial online] 2013 [cited 2021 Mar 7];58:160. Available from: https://www.e-ijd.org/text.asp?2013/58/2/160/108088

What was known? 1. Glomeruloid hemangioma is a specific marker for POEMS syndrome. 2. Few cases of glomeruloid hemangioma without POEMS syndrome have been reported in the literature.

   Introduction Top

Chan et al.[1] has coined the term 'glomeruloid hemangioma' for a histologically distinctive, cutaneous, benign vascular tumor, which is considered to be a specific marker for the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (POEMS) syndrome. Clinically, it is characterized by a solitary or multiple blue-red papules and histologically it resembles renal glomeruli. The POEMS syndrome is a multisystem disease comprising of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy (M-protein), and skin changes. Contrary to what has been previously believed, glomeruloid hemangioma has recently been reported without any evidence of the POEMS syndrome. [2],[3],[4],[5] We support the observation with two similar cases.

   Case Reports Top

Two young men, of age 18 and 22 years, presented with asymptomatic papulonodules of six months' and ten years' duration, respectively. One case had slowly progessing, multiple, pale, skin-colored papules and nodules present over the chin [Figure 1]. They were firm in consistency, varying in size from 1-2 cm. Few of the papular lesions were translucent in appearance and one of the papules had a 'nipple-like' projection. The lesions were non-compressible and non-tender. The second case had multiple, skin-colored, dome-shaped papules and nodules, present over the anterior and lateral aspect of neck, varying in size from 2 mm to 2 cm [Figure 2]. Systemic examination, including a neurological examination, did not reveal any abnormality.

Both underwent routine laboratory tests, which revealed no abnormality. No Bence-Jones proteinuria was detected in a sample from a 24-hour urine collection. Serum protein electrophoresis was normal. Abdominal ultrasound was normal and endocrinological evaluation, comprising of thyroid function tests, serum estradiol, serum testosterone, serum prolactin, and serum cortisol were all within normal range. A nerve-conduction velocity study and a total body skeletal survey were normal. Both cases had no abnormal findings on chest X-ray or computed tomography (CT) scans.
Figure 1: Skin - colored papules and nodules on the chin, with a 'nipple - like' projection

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Figure 2: Skin - colored, dome - shaped papules and nodules on the neck

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The histopathalogical examination revealed numerous dermal ectatic vascular spaces lined by flat endothelial cells. Inside these dilated vascular spaces, a conglomeration of capillaries lined by plump, swollen endothelial cells, filled with red blood cells, resembling renal glomeruli, were seen [Figure 3] and [Figure 4]. Variable-sized, homogeneous, eosinophilic, periodic acid-Schiff (PAS)-positive, diastase-resistant globules were present in the endothelial and stromal cells. The endothelial cells were positive for CD31 and CD34.
Figure 3: Photomicrograph showing glomeruloid aggregates of vascular structures in the dermis, resembling renal glomeruli (H and E, ×40)

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Figure 4: Photomicrograph showing a glomeruloid structure with dilated capillaries lined by plump endothelial cells, filled with red blood cells (H and E, ×400)

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   Discussion Top

Glomeruloid hemangiomas represent vascular tumors, which present as small, firm, red-to-violaceous, dome-shaped papules, papulonodules, subcutaneous bluish compressible tumors, wine-red sessile or pedunculated papules, or lesions with cerebriform morphology. They range in size, measuring few millimeters to few centimeters in diameter, and are located mainly on the trunk and proximal limbs. [6],[7] Rarely, glomeruloid hemangioma involving the face has also been reported. [8],[9] Eruptive angiomatous lesions, similar to eruptive histiocytoma, have also been described. [10]

Histogenesis of glomeruloid hemangioma is still obscure. It probably represents a reactive endothelial proliferation, wherein, the overproduction of the vascular endothelial growth factor (VEGF), IL-6, IL-1β, tumour necrosis factor-α, and estrogens are believed to be responsible for angioma formation. [1] Hemangiomas in the POEMS syndrome fall into four histopathological categories: Microvenular hemangiomas, cherry hemangiomas, [11] multinucleated cell angiohistiocytomas, [12] and glomeruloid hemangiomas. [1] These histopathological features may be seen in different areas of the same biopsy specimen. They have been considered by some authors, to represent different stages in the development of the same lesion, which exhibit different degrees of endothelial proliferation, in response to angiogenic stimuli. [8],[13] Tufted angioma is stated to be the most immature form of angiomatous lesions in the POEMS syndrome. [14]

A vascular lesion may appear well before the full blown POEMS syndrome develops, and therefore, a thorough evaluation and follow-up of patients with glomeruloid hemangioma, for potential development of this syndrome, is still recommended. The first case that was followed up for one year did not show any features of the POEMS syndrome and the other case was lost to follow-up.

   References Top

1.Chan JK, Fletcher CD, Hicklin GA, Rosai J. Glomeruloid hemangioma: A distinctive cutaneous lesion of multicentric Castleman's disease associated with POEMS syndrome. Am J Surg Pathol 1990;14:1036-46.  Back to cited text no. 1
2.Vélez D, Delgado-Jiménez Y, Fraga J. Solitary glomeruloid hemangioma without POEMS syndrome. J Cutan Pathol 2005;32:449-52.  Back to cited text no. 2
3.Piña-Oviedo S, López-Patiño S, Ortiz-Hidalgo C. Glomeruloid hemangiomas localized to the skin of the trunk with no clinical features of POEMS syndrome. Int J Dermatol 2006;45:1449-50.  Back to cited text no. 3
4.González-Guerra E, Haro MR, Fariña MC, Martín L, Manzarbeitia L, Requena L. Glomeruloid hemangioma is not always associated with POEMS syndrome. Clin Exp Dermatol 2008;34:800-3.  Back to cited text no. 4
5.Forman SB, Tyler WB, Ferringer TC, Elston DM. Glomeruloid hemangiomas without POEMS syndrome: Series of three cases. J Cutan Pathol 2007;34:956-7.  Back to cited text no. 5
6.Puig L, Moreno A, Domingo P, Llistosella E, de Moragas JM. Cutaneous angiomas in POEMS syndrome. J Am Acad Dermatol 1985;12:961-4.  Back to cited text no. 6
7.Chan PT, Lee KC, Chong LY, Cheung YF. Glomeruloid hemangioma with cerebriform morphology in a patient with POEMS syndrome. Clin Exp Dermatol 2006;31:775-7.  Back to cited text no. 7
8.Tsai CY, Lai CH, Chan HL, Kuo TT. Glomeruloid hemangioma - A specific cutaneous marker of POEMS syndrome. Int J Dermatol 2001;40:403-6.  Back to cited text no. 8
9.Yang SG, Cho KH, Bang YJ, Kim CW. A case of glomeruloid hemangioma associated with multicentric Castleman's disease. Am J Dermatopathol 1998;20:266-70.  Back to cited text no. 9
10.Perniciaro C. POEMS syndrome. Semin Dermatol 1995;14:162-5.  Back to cited text no. 10
11.Dereure O, Guillot B, Dandurand M, Bataille R, Chertok P, Donadio D, et al. Cutaneous lesions in POEMS syndrome. Apropos of 3 case reports and a review of the literature. Ann Dermatol Venereol 1990;117:283-90.  Back to cited text no. 11
12.Del Río R, Alsina M, Monteagudo J, Torremorell D, González U, Luelmo J, et al. POEMS syndrome and multiple angioproliferative lesions mimicking histiocytomas. Acta Derm Venereol 1994;74:388-90.  Back to cited text no. 12
13.Marina S, Broshtilova V. POEMS in childhood. Pediatr Dermatol 2006;23:145-8.  Back to cited text no. 13
14.Rongioletti F, Gambini C, Lerza R. Glomeruloid hemangioma: A cutaneous marker of POEMS syndrome. Am J Dermatopathol 1994;16:175-8.  Back to cited text no. 14

What is new? 1. Glomeruloid hemangioma may have a varied clinical presentation, as seen in our cases. 2. Glomeruloid hemangioma presenting with a ′nipple like′ projection has not been reported to our knowledge. 3. Clinicians require a high index of suspicion to diagnose glomeruloid hemangioma without the POEMS syndrome.


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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