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Year : 2016  |  Volume : 59  |  Issue : 3  |  Page : 392-394
Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood

1 Department of Pathology, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India
2 Department of Plastic Surgery, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India
3 Department of Pathology, St. Luke's Hospital, Milwaukee, WI 53215, USA

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Date of Web Publication10-Aug-2016


Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach–Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.

Keywords: Cutaneous kaposiform hemangioendothelioma, multiple, recurrent

How to cite this article:
Atla B, Sudhakar P V, Rao N, Prasad U. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood. Indian J Pathol Microbiol 2016;59:392-4

How to cite this URL:
Atla B, Sudhakar P V, Rao N, Prasad U. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood. Indian J Pathol Microbiol [serial online] 2016 [cited 2022 Sep 25];59:392-4. Available from:

   Introduction Top

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of skin and deep soft tissue typically first seen in infancy although it can also affect older children and adults. The designation of “hemangioendothelioma” is indicative of its somewhat uncertain biological behavior. Although it is locally aggressive, without metastatic potential, it may be associated with the potentially life-threatening Kasabach–Merritt syndrome (KMS), which is defined by thrombocytopenia with or without coagulopathy, as well as lymphangiomatosis.[1] The presence or absence of KMS has an important bearing on the prognosis of this condition.

   Case Report Top

A 3-year-old female child initially presented with a raised, light brown, plaque-like lesion measuring 1 cm, over the dorsum of the nose [Figure 1]a. A hemogram performed during the work-up was normal; in particular, the platelet count was within normal limits (230,000/cumm). A simple excision of the lesion was performed, and an initial histopathological diagnosis of hemangioma was rendered. Approximately, 6 months after initial presentation, the patient presented with a local recurrence. Over the next few years, there were multiple local recurrences, which were excised. At age 7, the patient presented with a large local recurrence measuring 4 cm × 3 cm, distorting the nose. In addition, a second swelling measuring 6 cm × 4 cm had appeared on the chin. The child underwent plastic reconstruction of the nose with a forehead flap and excision of the chin lesion.
Figure 1: (a) Initial presentation of the patient was a light brown plaque-like lesion on the dorsum of the nose. (b) Low power examination highlighting the nodular architecture and infiltrative margins into adjacent fibroadipose tissue (H and E, ×100). (c) High power examination highlighting slit-like vascular channels lined by plump and spindle cells and frequent red cell aggregates within vascular lumina. Cytological atypia is minimal, and mitotic activity is virtually absent (H and E, ×400). (d) Immunohistochemistry revealing strong and diffuse CD31 positivity of lesional cells

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Histological examination of both lesions showed similar features. There was subcutaneous involvement by irregular nodules of round to spindle-shaped cells separated by irregular fibrous bands, pushing into adjacent fibroadipose tissue [Figure 1]b. A few ectatic vessels were present at the periphery. Higher power examination revealed a dense proliferation of ill-formed vascular channels lined by spindle cells, low mitotic activity with minimal cytological atypia, and intravascular red cell aggregates, scattered microthrombi, and associated hemosiderin pigment [Figure 1]c.

An extensive immunohistochemical panel showed positivity of the lesional cells with vascular markers factor VIII, CD31, CD34, and Fli 1 [Figure 1]d. Myogenic markers muscle-specific actin and smooth muscle actin stained smooth muscle in vessel walls but were negative in lesional cells. Other markers including desmin, glucose transporter 1 (GLUT-1), and human herpes virus-8 (HHV-8) were also negative. The morphological appearances and the immunoprofile led to a histopathological diagnosis of KHE.

A small lesion was noted near the inner canthus of the left eye on a recent visit, which was histopathologically confirmed to be KHE. Careful systemic clinical and laboratory evaluation did not show evidence of thrombocytopenia. Serial hemograms revealed platelet counts ranging from 200,000 to 250,000/cumm. The patient did not undergo any other evaluation for coagulopathy. Of note, she has not so far been treated with radiation or chemotherapy. Most recently, she presented with a plaque-like lesion in the oral cavity with difficulty in swallowing solids.

   Discussion Top

KHE represents a spectrum of vascular neoplasia ranging from small superficial lesions without systemic manifestations, to large infiltrative tumors with life-threatening complications resulting from consumptive coagulopathy (KMS). The prevalence of KHE is 0.91/100,000 children.[2] Most patients with KHE present in infancy and childhood with classic cutaneous lesions. However, many patients may present with noncutaneous manifestations. They tend to be older and present with atypical signs and symptoms. Rare cases are seen in adulthood. Mentzel et al.[3] reported three cases occurring in adults, presenting at 64, 55, and 48 years, respectively.

The tumor has been reported to occur in varied anatomic locations including the head and neck,[4],[5],[6],[7] trunk, extremities, mediastinum, and retroperitoneum.[2]

Superficial lesions involve the dermis, deep fascia, and subcutaneous tissue. Deep lesions can involve skeletal muscle and bone, as well as intrathoracic or retroperitoneal sites.

KHE can be mistaken for several other vascular lesions with similar age distribution, but with different clinical course, management options, and prognosis. As such, careful histopathological examination and immunohistochemistry are imperative, as they hold the key to accurate diagnosis. Significant histological features include the presence of ill-defined, coalescent nodules with an admixture of predominantly small round to spindle-shaped cells, capillary sized, slit-like blood vessels, broadbands of collagen, and a scattering of inflammatory cells infiltrating adjacent soft tissue. Intracytoplasmic vacuolations, fragmented red blood cells, hyaline globules, hemosiderin pigment, and microthrombi are other findings. Mitotic figures are inconspicuous and nuclear atypia is minimal.

The histologic differential diagnosis includes several other types of vascular lesions of variable prognosis and significance. Juvenile hemangioma affects patients in the same age group as KHE. It has a polypoid red appearance grossly, as opposed to the plaque-like lesions of KHE. While it may share the nodular architecture of KHE, with round to oval cells lining capillary-sized blood vessels; spindling, hemosiderin pigment, fragmented red cells, and tumor infiltration into surrounding tissue are usually not seen. GLUT-1 immunostaining is very useful, as it tends to be positive in juvenile hemangioma and not in KHE (as demonstrated in our case). Although acquired tufted angioma can bear striking resemblance to KHE, careful attention to histological detail, in particular, infiltrative margins in the latter, should allow their separation. Spindle cell hemangioendothelioma has large cavernous spaces lined by flattened endothelial cells containing erythrocytes and thrombi, with an intervening benign appearing spindle cell proliferation. Calcific phleboliths are frequently present. Kaposi sarcoma may show many of the features of KHE including vasoformative spindle cells arranged in fascicles with slit-like lumina, microhemorrhages, and striking inflammatory infiltrate at the periphery. However, the absence of infiltrative margins and positive immunostaining with HHV-8 are useful in distinguishing it from KHE.

Although there are no specific criteria to risk stratify patients with respect to occurrence or recurrence of KMS; in general, superficial tumors, lesions isolated to bone or presentation at an older age are associated with decreased frequency of KMS. Tumors >8 cm, retroperitoneal (comprising 12–18% of KHE)[2],[8] and mediastinal tumors tend to be infiltrative and are associated with higher risk for KMS. Infiltrative KHE involving multiple anatomic sites (as in our case), regardless of size, also confer an increased risk of KMS. The consumptive coagulopathy which characterizes KMS is multifactorial. Thrombocytopenia in KMS (clinically significant thrombocytopenia - platelet count <30,000/µl) occurs from abnormal platelet activation and aggregation and is not due to a defect in platelet production. Small, convoluted capillaries and poorly formed discontinuous basal lamina of endothelial cells, permit interaction of platelets, and clotting factors with collagen, resulting in trapping of platelets and formation of microthrombi. KMS is refractory to transfused platelets, often causing painful tumor engorgement due to trapping of platelets.[2] In our case, the platelet counts were consistently within normal limits.

Behavior of this neoplasm is strongly influenced by site of origin, clinical extent, and development of KMS. In Fernand Mac-Moune Lai et al.'s [8] study of five patients with cutaneous lesions and follow-up ranging from 8 to 35 years (mean - 19 years), patients were treated with radiation or interferon therapy, and all patients were alive although three of them had persistent disease. Two patients had reached adulthood. Alpha-2A interferon therapy has been shown to have promise in the treatment of KHE, in particular, those who develop KMS. It is thought to interfere with angiogenesis in these patients by various mechanisms including blocking endothelial cell motility and proliferation. In patients with KMS, increased endothelial prostacyclin production and release appears to reduce platelet adherence and trapping.[9] Our patient's clinical course has been characterized by multiple local recurrences and appearance of new regional lesions, without distant metastases. Judging by the clinical extent of the disease, it would appear that there may be some risk for development of KMS although she has fortunately not so far developed the complication.

   Conclusion Top

KHE is an unusual vascular tumor primarily occurring in infancy and childhood. While it is generally indolent and protracted, with local and regional recurrences, but without distant metastases, the clinical course can be adversely affected by the serious complication of KMS. This report highlights a case of multiple superficial recurrent KHE without associated KMS. Our case emphasizes the importance of recognizing the histological features and use of appropriate immunohistochemistry that allow the subtle distinction from several other vascular lesions that can occur in the same age group but are characterized by a different clinical course and prognosis. Early and accurate diagnosis should allow treating physicians to prepare for the possibility of recurrence(s), and the potentially serious complication of KMS.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Enjolras O, Wassef M, Mazoyer E, Frieden IJ, Rieu PN, Drouet L, et al. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997;130:631-40.  Back to cited text no. 1
Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, et al. Kaposiform hemangioendothelioma: Atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013;162:142-7.  Back to cited text no. 2
Mentzel T, Mazzoleni G, Dei Tos AP, Fletcher CD. Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases. Am J Clin Pathol 1997;108:450-5.  Back to cited text no. 3
Lee CH, Jaw TS, Yang SF, Wu DK. Kaposiform hemangioendothelioma arising from the maxillary sinus: A case report. Kaohsiung J Med Sci 2010;26:154-7.  Back to cited text no. 4
Chang JM, Kwon BJ, Han MH, Kang HS, Chang KH. Kaposiform hemangioendothelioma arising from the internal auditory canal. AJNR Am J Neuroradiol 2006;27:931-3.  Back to cited text no. 5
Kim DW, Chung JH, Ahn SH, Kwon TK. Laryngeal kaposiform hemangioendothelioma: Case report and literature review. Auris Nasus Larynx 2010;37:258-62.  Back to cited text no. 6
Wilken JJ, Meier FA, Kornstein MJ. Kaposiform hemangioendothelioma of the thymus. Arch Pathol Lab Med 2000;124:1542-4.  Back to cited text no. 7
Mac-Moune Lai F, To KF, Choi PC, Leung PC, Kumta SM, Yuen PP, et al. Kaposiform hemangioendothelioma: Five patients with cutaneous lesion and long follow-up. Mod Pathol 2001;14:1087-92.  Back to cited text no. 8
Deb G, Jenkner A, De Sio L, Boldrini R, Bosman C, Standoli N, et al. Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with alpha-2A interferon. Med Pediatr Oncol 1997;28:358-61.  Back to cited text no. 9

Correspondence Address:
Dr. Bhagyalakshmi Atla
Department of Pathology, Andhra Medical College, King George Hospital, Visakhapatnam - 530 002, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.188130

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