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Year : 1992  |  Volume : 58  |  Issue : 3  |  Page : 206-207

Association of haemangiopericytoma and urticaria

Correspondence Address:
J Majumdar

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This is a report of oral haemangiopericytoma with urticaria in an infant indicating a previously unrecognized association. The urticarial eruptions cleared after the initiation of chemotherapy for the primary oral lesion and reappeared extensively with cutaneous metastasis after the malignant growth became resistant to treatment.

Keywords: Haemangiopericytoma, Urticaria

How to cite this article:
Majumdar J, Basu S, Dutta S K, Roy J C. Association of haemangiopericytoma and urticaria. Indian J Dermatol Venereol Leprol 1992;58:206-7

How to cite this URL:
Majumdar J, Basu S, Dutta S K, Roy J C. Association of haemangiopericytoma and urticaria. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 Sep 19];58:206-7. Available from:

  Introduction Top

Haemangiopericytoma, a rare vascular neoplasm, may occur anywhere on the body but has a predilection for the head and neck region and lower extremities in children. [1],[2] This tumour originates from the perivascular contractile pericytes of Zimmermann [3] and histopathological differentiation between the benign and malignant variants have been unsuccessful. [4] Herein, we report a case of oral haemangiopericytoma with urticaria. This association is being reported for the first time.

  Case Report Top

A 3-month-old baby boy presented with a rapidly growing, firm, fleshy and ulcerated growth of 4 cm x 1.25 cm size, protruding from his mouth and extending to the hard palate. It was of 2 months duration, and was fixed to the palate and bled on touch. Recurrent urticarial wheals were also present. Apart from moderate anaemia, laboratory and radiological investigations were unremarkable. Biopsy from the oral lesion confirmed the diagnosis of haemangiopericytoma. The patient was treated with chemotherapy consisting of vincristine, adriamycin and cyclophosphamide as his parents refused surgery and radiotherapy. The initial response to this regime was satisfactory with diminution of the growth in size (3.5 cms x 1.2 cms) and with disappearance of urticaria. However, after 4 months of treatment, the oral lesion became resistant to chemotherapy with reappearance of the urticaria together with the development of multiple generalized subcutaneous nodules. Biopsy from the subcutaneous nodules was suggestive of haemangiopericytoma. The infant died after one week due to severe haemorrhage from his primary lesion.

  Comments Top

There are several reports of haemangiopericytoma metastasizing to the lungs, brain, lymph nodes, liver and intestine. [2],[3],[4] This is perhaps the only case of haemangiopericytoma with multiple skin metastases.

Chronic urticarial skin reactions are an uncommon manifestation of malignancies like carcinoma, lymphoma and myeloma. However only in a few of these conditions, the cause effect relationship between the two could be ascertained. [5] In our patient, the urticarial wheals decreased considerably with the decrease in the size of the primary oral lesion after the initiation of therapy, and reappeared again when the primary lesion became resistant to treatment. The urticaria associated with hemangiopericytoma, like other malignancies, may be due to a hypersensitivity reaction of some component of the neoplasm. [5]

  References Top

1.Kauffmann S L, Stout A P. Hamangio-pericytoma in children. Cancer 1960; 13: 695 - 710.  Back to cited text no. 1    
2.Enzinger F M, Smith B H. Hamangio­pericytoma : An analysis of 106 cases. Hum Pathol 1976; 7 : 61 - 82.  Back to cited text no. 2    
3.Stout AP, Murray MR. Hamangiopericytoma : A vascular tumor featuring Zimmermann's pericytes. Ann Surg 1942; 116 : 26 - 33.  Back to cited text no. 3    
4.McMaster M J, Soule EH, Ivins J. Hemangiopericytoma : A clinicopathologic study and long term follow - up of 60 patients. Cancer 1975; 36: 2232 - 44.  Back to cited text no. 4    
5.Haynes H A. Cutaneous manifestations of internal malignancy. In : Harrison's principles of internal medicine, vol 2 (Braunwald E, Isselbacher K J, Petersdorf R G, et al, eds), 1 1 th edn. New York: McGraw - Hill Book Company 1987; 1592.  Back to cited text no. 5    


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