Case Report: Cutaneous Pleomorphic Lymphangiosarcoma in a Dog Exhibiting Features of Human Composite Hemangioendothelioma

Background: Angiosarcomas are a broad category of vascular origin neoplasms that are poorly characterized in veterinary species. Lymphangiosarcoma (LAS) is an uncommon type of angiosarcoma reported in humans and canines arising from lymphatic endothelium. LAS can be differentiated from other angiosarcomas in dogs based on expression of Prospero-related homeobox gene-1 (PROX-1) or lymphatic vessel endothelial receptor-1 (LYVE-1). Composite hemangioendothelioma (CHE) is a rare angiosarcoma subtype described in people and characterized by a variable biologic behavior and infrequent metastasis. This variant of angiosarcoma histologically combines features of retiform hemangioendothelioma and epithelioid hemangioendothelioma. Information regarding the cytologic and histopathologic appearance and clinical course of dogs with vascular tumors that exhibit features of CHE are unknown. Here, we report a case of pleomorphic LAS with features of CHE arising in a dog and treated with surgery and adjuvant chemotherapy.Case presentation: A 10-year-old intact male Labrador retriever presented with an approximately 6-cm-diameter cutaneous mass caudal to the left elbow that was progressively growing over 1.5 years. On physical examination, palpable extensions were identified coursing proximally over the triceps with concurrent loco-regional peripheral lymphadenopathy. Fine needle aspirates (FNA) and cytologic assessment of the cutaneous mass, left prescapular, and accessory axillary lymph nodes reported that this appeared to be a metastatic epithelial neoplasm, although a mixed carcinoma or collision tumor could not be excluded. An incisional biopsy of the mass was submitted for histopathology and was consistent with a well-differentiated angiosarcoma with features of CHE. The neoplasm expressed vimentin, CD31, von Willebrand factor (vWf), and PROX-1, supporting the diagnosis of LAS. Complete staging was performed, and no additional metastatic lesions were identified. Left forelimb amputation and lymph node removal were performed. Based on the diagnosis of metastatic LAS, doxorubicin chemotherapy was administered. 7 months post-amputation, the tumor recurred at the amputation site without evidence of metastatic disease.Conclusion: This report describes a malignant, locally aggressive lymphatic origin vascular tumor in a dog, with features consistent with descriptions of CHE in humans. Cytologic features in this case were discordant with its true mesenchymal etiology, obfuscating diagnosis. The morphologic features of the mesenchymal neoplastic population and immunohistochemistry (IHC) labeling ultimately supported a diagnosis of pleomorphic LAS with features of CHE.

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Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease

BMC Oral Health ◽

10.1186/s12903-020-01291-4 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Gintaras Januzis ◽

Dovydas Sakalys ◽

Martynas Mantas Krukis ◽

Dmitrij Seinin

Keyword(s):

Correct Diagnosis ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Low Grade ◽

X Rays ◽

Adequate Treatment ◽

Clinical Appearance ◽

Allogenic Bone ◽

Bony Destruction

Abstract Background Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. Case presentation We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. Conclusions With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

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Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Oncology Reviews ◽

10.4081/oncol.2014.259 ◽

2014 ◽

Cited By ~ 68

Author(s):

Angela Sardaro ◽

Lilia Bardoscia ◽

Maria Fonte Petruzzelli ◽

Maurizio Portaluri

Keyword(s):

Poor Prognosis ◽

Therapeutic Option ◽

Correct Diagnosis ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Morphological Data ◽

Vascular Tumors ◽

Vascular Differentiation ◽

The Times ◽

One Year

Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

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Malignant Vascular Bone Tumors

Musculoskeletal Imaging Volume 1 ◽

10.1093/med/9780190938161.003.0061 ◽

2019 ◽

pp. 308-312

Author(s):

G. Scott Stacy

Keyword(s):

Bone Tumors ◽

Malignant Tumors ◽

Treatment Strategies ◽

Vascular Tumor ◽

Epithelioid Hemangioendothelioma ◽

Vascular Tumors ◽

Bone Lesions ◽

Imaging Features ◽

Vascular Bone Tumors ◽

Health Organization

The organization of vascular bone tumors can be thought of as a spectrum of disease including both benign and malignant entities. Chapter 61 focuses on the malignant tumors, which have elicited much controversy and debate. These include 2 vascular tumors of bone recognized by the World Health Organization: epithelioid hemangioendothelioma and angiosarcoma. Although there are no characteristic imaging features that would obviate the need for biopsy of these entities, they may present with multifocal bone lesions, which can suggest the diagnosis of a vascular tumor. This chapter will describe the clinical, pathophysiological, and imaging features of these malignant vascular bone tumors, with a brief mention of treatment strategies.

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A Unique Collision Tumor in Breast

Archives of Pathology & Laboratory Medicine ◽

10.5858/2004-128-99-auctib ◽

2004 ◽

Vol 128 (1) ◽

pp. 99-101

Author(s):

Barbara Susnik ◽

J. Jordi Rowe ◽

Philip N. Redlich ◽

Christopher Chitambar ◽

Chung-Che Chang ◽

...

Keyword(s):

Malignant Lymphoma ◽

Invasive Ductal Carcinoma ◽

Core Biopsy ◽

Ductal Carcinoma ◽

B Cell Lymphoma ◽

Medullary Carcinoma ◽

Collision Tumor ◽

Metastatic Carcinoma ◽

Axillary Lymph Nodes ◽

Axillary Lymph

Abstract We report an extraordinary case of a collision tumor consisting of invasive ductal carcinoma with adjacent malignant lymphoma presenting as a single mass in the breast. A 79-year-old woman presented with a breast mass. A core biopsy performed at an outside hospital was interpreted as medullary carcinoma. On review of the breast core biopsy, a diagnosis of a synchronous malignant lymphoma and invasive ductal carcinoma was rendered. The patient underwent lumpectomy and axillary dissection. The excised specimen revealed a 2.1-cm, moderately differentiated invasive ductal carcinoma, partially surrounded by malignant lymphoma with areas where both tumors were intermixed. All 27 axillary lymph nodes were extensively involved by lymphoma, and 1 lymph node demonstrated metastatic carcinoma. The morphology and results of immunohistochemistry, flow cytometry, and cytogenetic analysis were consistent with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue.

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