2
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Bifocal Tibial Kaposiform Hemangioendothelioma Responsive to Vincristine Therapy: A Case Report

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Patient: Female, 9-year-old

          Final Diagnosis: Kaposiform hemangioendothelioma

          Symptoms: Leg pain

          Medication: —

          Clinical Procedure: —

          Specialty: Orthopedics and Traumatology

          Objective:

          Rare co-existance of disease or pathology

          Background:

          Kaposiform hemangioendothelioma is a rare locally aggressive vascular endothelial-derived spindle cells neoplasm. Herein, we report a rare case of bifocal tibial kaposiform hemangioendothelioma.

          Case Report:

          A 9-year-old female presented with a 2-year history of pain and swelling in the left leg. The patient had a high plasma level of the D-dimer and fibrinogen. Radiography revealed a centric lytic lesion on the left proximal tibial metaphysis and an eccentric lateral distal tibial metaphyseal. Histopathologic examination of the sample taken from the distal tibia revealed a dense spindle cell tumor with lobular architecture composed of compact spindle cells compressing small slit-like vascular spaces, forming glomeruloid nests. No necrosis was identified. Based on these findings and the positive immunohistochemical staining for CD31, CD34, and D2-40, the patient was diagnosed with kaposiform hemangioendothelioma. Treatment was started by using vincristine chemotherapy, after which the patient developed temporary peroneal neuropathy, which improved over the next 3 months.

          Conclusions:

          Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. In these cases, the treating physician should be aware of peroneal neuropathy as a potential complication of vincristine administration.

          Related collections

          Most cited references20

          • Record: found
          • Abstract: found
          • Article: not found

          Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.

          Kaposiform hemangioendothelioma (KH) is a rare tumor of childhood often associated with Kasabach-Merritt phenomenon (KMP) and occasionally lymphangiomatosis. Although generally considered distinct from other vascular neoplasms, its rarity has precluded a thorough study of its immunophenotypic profile and long-term behavior. Thirty-three cases of KH were reviewed and immunostained for alpha-smooth muscle actin, various endothelial markers (CD31, CD34, vWf, FLI1), a platelet marker (CD61), and the juvenile hemangioma-associated markers GLUT-1 and Lewis Y antigen (LeY). In addition, the presence of HHV-8 was evaluated by RT-PCR. The patients (20 males and 13 females) ranged in age from 2 weeks to 20 years (mean 3 years 9 months). Tumors developed on the extremities (17 cases), head/neck (8 cases), and other sites (8 cases) and affected both superficial and deep soft tissue. Those in the skin presented as slightly raised blue-red lesions. More than half of the patients presented with KMP (14 of 25). Tumors consisted of irregular, infiltrating nodules of compressed vessels, which modulated between areas resembling a capillary hemangioma and Kaposi sarcoma (KS). Endothelial cells in nodules were CD31, CD34, and FLI1 positive but negative for GLUT1 and LeY. Scattered "epithelioid" or glomeruloid islands featuring endothelium associated with clusters of plump alpha-smooth muscle actin-positive pericytes, stippled hemosiderin, and CD61-positive fibrin thrombi likely represent the morphologic sites of platelet consumption. Small and large lymphatic channels occurred in 22 of 33 cases and were typically seen peripheral or deep to the main tumor mass. HHV-8 transcripts were not identified (0 of 3 cases). Follow-up information was available in 22 patients (range 8 months to 15 years; mean 2 years) and indicated that 3 died of disease, 8 were alive with disease, and 10 were alive without residual disease. Two patients developed regional perinodal soft tissue involvement, but none developed distant metastases. KH is a lesion having both a vascular and lymphatic component. Its common association with KMP probably relates in part to unique architectural features that favor turbulent blood flow and platelet activation. KH can also be reliably separated from JH by GLUT-1 and LeY immunostaining, indicating differences in the morphologic and functional attributes of the endothelium between the two lesions. The absence of HHV-8 in KH underscores a different pathogenesis from Kaposi sarcoma. Our study, the largest to date, emphasizes that mortality is due to KMP and not metastatic disease, which appears limited to regional perinodal soft tissue. Given this behavior, its continued classification as a vascular tumor of intermediate malignancy is warranted.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma.

            Children with a large vascular tumor and associated Kasabach-Merritt coagulopathy respond inconsistently to therapy and have a high mortality rate. For this reason, we undertook a retrospective study of 21 such patients, and focused on clinical, radiographic, and histopathologic features. The male to female ratio was 1:1.6. Tumor was noted at birth in 50 percent of patients; the remainder appeared throughout infancy. The location was cervicofacial (n = 2), shoulder/upper limb (n = 4), trunk including retroperitoneum (n = 11), and lower limb (n = 4). These tumors grew rapidly to large size and were characterized by cutaneous purpura, edema, and an advancing ecchymotic margin. In contrast to common hemangioma, magnetic resonance imaging showed diffuse enhancement with ill-defined margins, cutaneous thickening, stranding of subcutaneous fat, hemosiderin deposits, and small feeding and draining vessels. All tumors were Kaposiform hemangioendothelioma (KHE); none were infantile hemangioma. Light microscopy showed irregular lobules or sheets of poorly formed, small vascular channels infiltrating and entrapping normal tissues. Characteristic features included spindle-shaped endothelial cells, diminished pericytes and mast cells, microthrombi, and hemosiderin deposits. Wide endothelial intercellular gaps and incomplete basement membranes were seen by electron microscopy. Dilated, hyperplastic, lymphaticoid channels were prominent in one tumor. KHE in 14 infants was treated with interferon alpha-2a: 6 had accelerated regression; 2 had stabilization of growth; and 6 evidenced no response. The mortality rate was 24 percent (5 of 21); this included three infants with retroperitoneal KHE. Kasabach-Merritt phenomenon does not occur with common hemangioma. Rather it is associated with the more aggressive KHE and rarely with other vascular neoplasms. Variable response to current pharmacologic therapy underscores our inadequate knowledge of the pathogenesis of thrombocytopenia in KHE.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis.

              We report the clinical and pathological features of nine distinctive, but relatively unknown, vascular tumors of infancy and childhood presenting as soft tissue masses often associated with locally aggressive disease, lymphangiomatosis and Kasabach-Merritt syndrome. The patients, four males and five females, were all in their first decade of life except for two (median, 2 years; range, 5 months to 19 years). These tumors involved deep soft tissues of the upper extremity (four cases), retroperitoneum (two cases), chest wall, scalp, and neck (one case each). Four patients also had Kasabach-Merritt syndrome, and three patients had lymphangiomatosis. Lymphangiomatosis consisted of diffusely infiltrating lymphangioma of soft tissue (three cases) and in two by the additional presence of bone lesions. In one of these three cases, lymphangiomatosis antedated the diagnosis of the vascular tumor, and in the remainder they were concurrently diagnosed. Tumors were characterized by infiltrating, interconnecting sheets or irregular nodules of slender endothelial cells lining crescentic or slit-like vessels and, less commonly, rounded capillary-type vessels. Within some tumors, nests of epithelioid endothelial cells with prominent eosinophilic cytoplasm containing finely granular hemosiderin, hyaline droplets, and cytoplasmic vacuoles were identified. Smaller amounts of hemosiderin were observed within the spindled endothelial cells and microthrombi could be seen occasionally within the tiny lumina. Nuclear atypia was minimal within these tumors and mitotic figures were infrequent, averaging 2 to 3/10 high-power fields (HPF) (range 0-7/10 HPF). Larger, well-formed feeding vessels were present at the periphery of the tumor. The endothelium of these vessels expressed factor VIII-AG, CD34, and bound Ulex europaeus, and contained an occasional perithelial cell expressing muscle-specific actin. In contrast, the spindled tumor cells expressed only CD34. Human papilloma virus (HPV)-16-like DNA transcripts, which have been identified in cases of Kaposi's sarcoma, were not detected by polymerase chain reaction in two cases. Follow-up information revealed that four patients were alive without disease after wide excision, multiple excision(s), or amputation (one case); three were alive with disease; and two died, one from lymphangiomatosis with respiratory compromise and the other from hemorrhage complicating Kasabach-Merritt syndrome. It appears that treatment should consist of wide local excision and supportive therapy for associated symptoms.
                Bookmark

                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2019
                23 December 2019
                : 20
                : 1923-1929
                Affiliations
                [1 ]Department of Anesthesia, School of Medicine, University of Jordan, Amman, Jordan
                [2 ]Department of General Surgery, University of Jordan, Amman, Jordan
                [3 ]Department of Special Surgery, School of Higher Studies, University of Jordan, Amman, Jordan
                [4 ]Department of Pathology, School of Medicine, University of Jordan, Amman, Jordan
                [5 ]Department of Internal Medicine, University of Jordan, Amman, Jordan
                [6 ]Department of Special Surgery, School of Medicine, University of Jordan, Amman, Jordan
                Author notes

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Conflict of interest: None declared

                Corresponding Author: Omar Q. Samarah, e-mail: o.samarah@ 123456ju.edu.jo
                Article
                917696
                10.12659/AJCR.917696
                6944038
                31866667
                c2fcb98f-19ce-423c-83ea-7de4feb884f7
                © Am J Case Rep, 2019

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 22 May 2019
                : 16 October 2019
                Categories
                Articles

                hemangioendothelioma,peroneal neuropathies,vincristine

                Comments

                Comment on this article