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CASE REPORT |
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| Year : 2007 | Volume
: 52
| Issue : 3 | Page : 160-162 |
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| Congenital retiform hemangioendothelioma |
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Savas Serel1, Belcin Izol Serel2, Arsin Uluc1, Aylin Okcu Heper3, Mehmet Serdar Gultan1
1 Department of Plastic, Reconstructive and Aesthetic Surgery, Medical School of Ankara University, Ankara, Turkey
2 Department of Dermatology, Ministry of Health Ankara Research and Training Hospital, Ankara, Turkey
3 Department of Pathology, Medical School of Ankara University, Ankara, Turkey
Correspondence Address:
Savas Serel
Department of Plastic, Reconstructive and Aesthetic Surgery, Medical School of Ankara University, Cebeci Hospital, 06590-Dikimevi, Ankara
Turkey
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-5154.35354
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 Abstract | | |
Background: Retiform hemangioendothelioma (RH) is an extremely rare and distinct variant of low-grade, well-differentiated cutaneous angiosarcoma seen in young adults and is characterized by a high recurrence rate, but low metastatic potential. To the best of our knowledge, this is the first report of the congenital RH. The lesion was treated by wide surgical resection. Biopsy revealed RH. We herein describe a congenital case of this rare vascular tumor, arising on the left foot, which was diagnosed when the patient was 21 years old. Results and Conclusions: There was no recurrence or lymph node metastasis during a follow-up period of 6 months. The authors expand the concept of RH by adding a congenital case.
Keywords: Congenital, retiform hemangioendothelioma
How to cite this article:
Serel S, Serel BI, Uluc A, Heper AO, Gultan MS. Congenital retiform hemangioendothelioma. Indian J Dermatol 2007;52:160-2 |
| Introduction | |  |
In 1994, Calonje et al . first described a series of 15 patients with retiform hemangioendothelioma (RH). [1] The term RH emphasizes its intermediate biologic behavior and histomorphologic resemblance to normal rete testis (retiform pattern). Since this original report, there have been no instances of congenital RH in the English literature to date. Preferential location sites are the extremities and trunk with only few isolated cases occurring on the head and penis. [1],[2] RH usually affects young and middle-aged adults and rarely occurs in infancy and elderly. There is a female predilection. The clinical picture does not provide help and the exact diagnosis therefore depends entirely on typical histopathologic features. [2] the treatment of choice is surgical excision with histopathologically tumor-free margins. [2]
We herein report a case of congenital RH affecting the dorsum of the foot of a 27-year-old male. To the best of our knowledge, this is the first case of congenital RH.
| Case Report | | |
A 27-year-old male was admitted to our clinic presenting with an ulcerated bleeding lesion. According to his medical history, it was first noted during the first month of his life, as a reddish brown nodule on the left foot. There was no clinical history of radiation exposure or radiotherapy and the family history was unremarkable. He could not have surgery because of insurance problems until this age. On physical examination, a bluish gray tumoral lesion, 11×15cm in diameter was observed and it extended from the ankle to the dorsal surface of the toes with focal bleeding areas and nodule formations [Figure - 1]. Laboratory examinations, including the blood cell counts, urinalysis, routine blood chemistry, immunologic investigations and endocrine screening, were within the normal ranges or negative. There was no clinical evidence of the regional lymph-node involvement. Magnetic resonance imaging showed the lesion extending from the dorsal surface of the second toe to the medial surface of the fourth toe of the left foot and in the plantar region as far as the second, third and fourth metatarsalphalangeal joints [Figure - 2].
Complete surgical excision was performed with a margin of at least 1 cm, removing the soft tissue down to the tendon fascia and periosteum [Figure - 3]. The second toe was resected up to the metatarsalphalangeal joint. The lesions on the third and the fourth toe were excised down to the fascia and periosteum and were grafted since the patient had not accepted the amputation of these fingers. Tissue loss on the dorsum of the foot was repaired by a free anterolateral thigh perforator flap [Figure - 4]. No radiation or chemotherapy was applied. The postoperative period was uneventful. The patient was discharged two weeks after the operation. There was no recurrence or lymph node metastasis during the follow-up period of 6 months [Figure - 5].
Histopathology
Histological examination of the excision materials revealed an extensive and ill-defined tumor consisted with numerous, monotoneous elongated vessels resembling the rete testis. The vessels were embedded in a wide collagenous stroma, and they were lined by single cuboidal hobnail endothelial cells with high nuclear/cytoplasmic ratio and occasionally the nuclear groove. Scattered lymphocytic inflammatory infiltration that was arranged in small groups in the stroma was noted. There was very rare small intraluminar papillary tufting. No significant atypia or mitotic figures were detected. Immunohistochemically, the tumor cells lining vessels reacted with CD31 and CD34. They were negative with HHV-8 [Figure - 6],[Figure - 7].
| Discussion | | |
RH, a rare and recently recognized vascular neoplasm, was originally described by Calonje and his colleagues. [1],[3] Morphologically, the lesions are either in the form of a single slowly enlarging exophytic mass or a plaque-like dermal and subcutaneous tumor in varying sizes. It has no distinctive clinical appearance: it may occur in the form of a slowly growing exophytic mass, a plaque-like lesion or a dermal and subcutaneous nodule. Such a clinical presentation is misleading and does not suggest the vascular nature of the tumor. [4] The differential diagnosis of RH includes a number of vascular tumors. Preferential location sites are the extremities and trunk with only few isolated cases occurring on the head and penis. [1],[2],[5],[6],[7] There is a female predilection.
RH and Dabska (papillary intralymphatic angioendothelioma) tumors are two closely related tumors and both of them are characterized by vascular proliferation lined by a single layer of hobnail endothelial cells. The common term "Hobnail Hemangioendothelioma" was proposed by some authors attributed to their similarities such as extra- and intravascular stromal lymphocytic inflammatory infiltration, perivascular stromal hyalinization in addition to the hobnail endothelial cells. [8] Both the tumors are located in the dermis and subcutaneous tissue of the extremities, particularly the lower limb. [9] Unlike Dabska tumor, retiform architecture is the dominant feature of the RH and intraluminal papillary projections are more prominent and lymphangiectatic vessels in Dabska tumor. [8] In contrast to their microscopic similarities, the epidemiology is different between these two tumors. Dabska tumor has predilection to infants and children whereas RH is common in young adults. [8],[9] Our case is unique with microscopic features of RH on unusual infantile clinical settings. The most important microscopic differential diagnosis of RH is angiosarcoma. The latter shows cytological atypia, mitosis, dissection individual collagen bundles and absence of hobnail endothelial cells; all these were absent in both in our case.
The treatment of choice is surgical excision with histopathologically tumor-free margins. Radiotherapy has also been proven effective in cases with lymph node metastasis, extended tumor size or local recurrence. [2],[3],[4],[6],[7] It is apparent that the occurrence of RH in the case of a patient with other neoplasms should always be the subject of further investigation in order to disclose a disease evolution.
RH usually affects the young and middle-aged adults and rarely occurs in infancy and elderly. [2],[3],[6] The age of the reported cases ranged from 9 to 78 years. [1],[3],[4],[6] Since the original report, there has been no congenital RH in the English literature to date. To the best of our knowledge, this is the first report of the congenital RH. This case points out that RH as a distinct entity can also be congenitally observed.
| References | | |
| 1. | Ioannidou D, Panayiotides J, Krasagakis K, Stefanidou M, Manios A, Tosca A. Retiform hemangioendothelioma presenting as bruise-like plaque in an adult woman. Int J Dermatol 2006;45:53-5.  |
| 2. | Reis-Filho JS, Paiva ME, Lopes JM. Congenital composite hemangioendothelioma: Case report and reappraisal of the hemangioendothelioma spectrum. J Cutan Pathol 2002;29:226-31. |
| 3. | Biagioli M, Sbano P, Miracco C, Fimiani M. Composite cutaneous aemangioendothelioma: Case report and review of the literature. Clin Exp Dermatol 2005;30:385-7. |
| 4. | El Darouti M, Marzouk SA, Sobhi RM, Bassiouni DA. Retiform hemangioendothelioma. Int J Dermatol 2000;39:365-8. |
| 5. | Tan D, Kraybill W, Cheney RT, Khoury T. Retiform hemangioendothelioma: A case report and review of the literature. J Cutan Pathol 2005;32:634-7. |
| 6. | Floris G, Deraedt K, Samson I, Brys P, Sciot R. Epithelioid hemangioma of bone: A potentially metastasizing tumor? Int J Surg Pathol 2006;14:9-15. |
| 7. | Calonje E, Fletcher C, Wilson-Jones E, Rosai J. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25. |
| 8. | Weiss SW, Goldblum JR. Hobnail (Dabska-Retiform) Hemangioendotelioma. In : Enzinger and Weiss's Soft Tissue Tumors. 4 th ed. Mosby: St. Louis; 2001. p. 906-13. |
| 9. | Colonje E. Retiform hemangioendotelioma, papillary intralymphatic angioendothelioma. In : WHO Classifications of tumors. Pathology and genetics, Tumors of soft tissue and bone. Fletcher C, Unni KK, Mertens F, editors. 2002. p. 165- |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7] |
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