Clinicopathological features of congenital hemangioma: a study of 40 cases / 中华病理学杂志

Objective: To investigate the clinical and pathologic features, diagnosis and differential diagnosis of congenital hemangioma (CH). Methods: Forty cases of CH were diagnosed from January 2017 to December 2020 in Henan Provincial People's Hospital. The clinical and pathological and immunohistochemical data were analyzed, with review of literature. Results: There were 24 male and 16 female patients. The lesions were located in the head, neck (11 cases), limbs (14 cases), and trunk (15 cases). The clinical manifestations were congenital painless plaques or masses, the larger ones protruded on the skin surface, mostly dusky purple or bright red, with surrounding white halos. Under low magnification, the tumor was lobular and well demarcated, composed of neo-microvascular lumen of different sizes. The vascular endothelial cells were cuboidal or hobnail in appearance, forming stellar drainage vessels within the lobules. Extra-medullary hematopoiesis was seen in one case of rapidly involuting CH; there were different number of tortuous and dilated vascular lumen between the lobular structures, and some non-involuting CH cases were vascular malformations, which were devoid of lobulated structures. Immunohistochemistry showed that endothelial cells were strongly positive for CD31, CD34 and ERG, while D2-40 and GLUT-1 were negative. Conclusions: CH is a benign congenital vascular tumor with characteristic lobulated growth and abnormal blood vessels in the stroma. Pathological diagnosis often needs to be differentiated from infantile hemangioma, pyogenic granuloma, kaposiform hemangioendothelioma and vascular malformation.

Papillary intralymphatic angioendothelioma: Dabska tumor

Abstract Papillary intralymphatic angioendothelioma (Dabska tumor) is a rarely metastasizing lymphatic vascular neoplasm that usually affects children and young adults. The majority of these cases occur in soft tissues of extremities, and to date less than 40 cases have been described. Despite the generally indolent evolution, can be locally invasive with the potential to metastasize. We describe a case of a young woman presenting with a plantar lesion, for 9 months and histological diagnosis of Dabska tumor. This neoplasm should be considered in the differential diagnosis of vascular dermatoses, allowing early diagnosis and treatment. Long-term follow-up should be performed.

Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma / Primary malignant hepatic tumors other than hepatocarcinoma or cholangiocarcinoma: a series of cases

Background: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. Aim: To review a registry of liver biopsies performed to diagnose hepatic tumors. Patients and Methods: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. Results: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. Conclusions: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

Hiperplasia endotelial papilar intravascular en la cavidad oral: reporte de un caso / Intravascular papillary endothelial hyperplasia: report of a case

La hiperplasia endotelial papilar intravascular (HEPI) es una lesiónbenigna muy poco común en la cavidad oral. Fue descrita por primera vez por Masson en 1923 como hemangioendotelioma vegetante intravascular; más adelante, se le dieron diferentes nombres como angiomatosis intravascular, seudoangiosarcoma de Masson y, por último, hiperplasia endotelial papilífera intravascular. Los hallazgos en bocason poco comunes, y cuando se presentan, generalmente es en labio.Se expone el caso clínico de un paciente de 78 años de edad con una lesión asintomática bilobular en encía, que refi ere una evolución lenta y progresiva de 10 años. Se emitió un diagnóstico clínico presuntivo degranuloma periférico de células gigantes y se procedió a realizar biopsiaescisional, así como su envío a estudio histopatológico para confirmar eldiagnóstico; se emitió el diagnóstico de HEPI y se confirmó por medio de inmunohistoquímica. Esta lesión puede ser fácilmente confundida con neoplasias malignas vasculares (angiosarcoma, sarcoma de Kaposi),tanto clínica como histopatológicamente. El objetivo de este trabajo es describir mediante un caso clínico la importancia de un correcto diagnóstico a través de una adecuada exploración clínica, así comouna observación microscópica a conciencia y la comunicación interdisciplinaria entre el cirujano maxilofacial y el patólogo bucal, para no confundir esta entidad benigna con otro tipo de patologías que requieren tratamientos más agresivos e invasivos, sin ser adecuados para la HEPI.

Intravascular papillary endothelial hyperplasia (IPEH) is a rarebenign lesion in the oral cavity. It was fi rst described by Massonin 1923 as intravascular vegetating hemangio endothelioma, andlater received diff erent names, like «intravascular angiomatosis¼,«Masson’s pseudoangiosarcoma¼ and «intravascular papillaryendothelial hyperplasia¼; fi ndings in the mouth are rare, and when present, they are generally in the lip. The clinical case presented is a78-year-old patient with a bilobular asymptomatic lesion in gingivawith a slow and progressive 10-year evolution. A presumptive clinicaldiagnosis of peripheral giant-cell granuloma was issued, followed byan excisional biopsy and histopathology study to confi rm the diagnosis;it had been correctly diagnosed as IPEH, which was confi rmed byimmuno histochemistry. This lesion can be easily confused with vascular malignancies (angiosarcoma, Kaposi sarcoma) both clinically andhistologically. The intention of this work is, through a clinical case,to describe the importance of a correct diagnosis by a proper clinical examination, detailed microscopic observation and interdisciplinary communication between the surgeon and the pathologist, in order toavoid a misdiagnosis of this benign entity with other pathologies tha trequire more aggressive and invasive treatments, inadequate for IPEH.

Exome sequence analysis of Kaposiform hemangioendothelioma: identification of putative driver mutations

Abstract BACKGROUND: Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments. OBJECTIVE: In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. METHOD: The genomic DNA for exome sequencing was obtained from the tumor tissue and matched normal tissue from the same individual. Exome sequencing was performed on HiSeq2000 sequencer platform. RESULTS: Among oncogenes, germline missense single nucleotide variants were observed in the TP53 and APC genes in both the tumor and normal tissue. As tumor-specific somatic mutations, we identified 81 candidate genes, including 4 nonsense changes, 68 missense changes and 9 insertions/deletions. The mutations in ITGB2, IL-32 and DIDO1 were included in them. CONCLUSION: This is a pilot study, and future analysis with more patients is needed to clarify: the detailed pathogenesis of this tumor, the novel diagnostic methods by detecting specific mutations, and the new therapeutic strategies targeting the mutation.

Hemangioendotelioma epitelioide y fusiforme de ganglio linfático: Caso clínico / Spindle and epithelioid hemangio-endothelioma of the lymph node: Report of one case

Background: Primary vascular tumors of lymph nodes are extremely rare with the exception of AlDS-related Kaposi's sarcoma. The diagnosis of epithelioid hemangio-endothelioma (EH) is difficult to make without ancillary studies, since it is devoid of morphological features indicating its vascular nature and it may be overlooked when it appears as a primary tumor of lymph nodes. Spindle and epithelioid hemangio-endothelioma (SEH) is considered to be a variant of EH, which has been reported to occur exclusively in lymph nodes and the spleen. We report a 70-year-old male with chronic lymphocytic leukemia (CLL) and left cervical lymphadenopathy. An excisional biopsy was performed, and microscopically the lymph node showed effacement of nodal architecture by a tumor composed of spindle cells disposed in intersecting fascicles, and characterized by abundant eosinophilic cytoplasm, elongated nuclei and conspicuous nucleoli. A second population of cells had an epithelioid appearance with intracyto-plasmic vacuoles containing red blood cells. lmmunohistochemically, the tumor cells were positive for CD31 and CD34. The final diagnosis was SEH of the lymph node.

Epithelioid haemangioendothelioma of the palate

Epithelioid Haemangioendothelioma [EHE] is an uncommon malignant vascular neoplasm that can develop at any anatomical site. In our report we describe a case of oral Epithelioid Haemangioendothelioma. A 13 years old male boy presented in the Oral Surgery Department of Armed Forces Institute of Dentistry with a growth in the left hard palate for the past 3 weeks. The growth was removed surgically and the specimen was sent to the Histopathology department of Armed Forces Institute of Pathology. On the basis of morphological features it was diagnosed as Epithelioid Haemangioendothelioma of the palate

Kasabach-Merritt syndrome: a case report.

Kasabach-Meritt syndrome is a combination of thromobocytopenia, hemolytic anemia, and acute or chronic consumptive coagulopathy in association with rapidly enlarging hemangioma. A male infant of 5 days was admitted in paediatric ward with this syndrome. The baby had ecchymotic patches over face and extremities and bleeding through umbilical stump. The child expired due to severe thrombocytopenia with consumptive coagulopathy leading to precipituous hemorrhage superimposed by septicemia. An autopsy was performed which confirmed retroperitoneal lesion as kaposiform hemangioendothelioma.

Intravascular Papillary Endothelial Hyperplasia (Masson's Hemangioma) of the Liver: A New Hepatic Lesion

Intravascular papillary endothelial hyperplasia (Masson's hemangioma) is a disease characterized by exuberant endothelial proliferation within the lumen of medium-sized veins. In 1923, Masson regarded this disease as a neoplasm inducing endothelial proliferation, however, now it is considered to be a reactive vascular proliferation following traumatic vascular stasis. The lesion has a propensity to occur in the head, neck, fingers, and trunk. Occurrence within the abdominal cavity is known to be very rare, and especially in the liver, there has been no reported case up to date. The authors have experienced intravascular papillary endothelial hyperplasia of the liver in a 69-yr-old woman, and report the case with a review of the literature.

Epithelioid and retiform hemangioendothelioma of the skull bone--report of four cases.

Hemangioendothelioma (HE) is a borderline or intermediate type of vascular neoplasm. We report clinical and histopathological characteristics of four cases of HE arising from the skull bones because of its extreme rarity in this location. The age of the patients ranged from 6-45 years. Three patients presented with a painless swelling over the head and one case had sphenoid wing mass with dimness of vision and proptosis. Radiographic images showed a well-demarcated, osteolytic lesion in the skull bone in all the four, one case in addition had sclerotic edges and another had specks of calcification. Grossly, the tumour was very vascular with hemorrhagic areas. Histologically, three cases showed features of an epithelioid variant of HE, with short strands and solid nests of rounded to slightly spindled, eosinophilic endothelial cells, some of them having small intracellular vacuoles. The stroma was myxoid--hyalinised with focal mixed inflammatory infiltrate. One case had features of a 'retiform' histological variant composed of numerous elongated vessels lined by a single layer of hobnail endothelial cells, focal lymphocytic infiltrate and papillae with hyaline collagenous cores. The tumour cells in all the four were immuno-labelled by antibody to factor VIII-associated protein. The tumour cells lacked cytological atypia and mitosis was sparse. These features were important in prognostication as low-grade tumours can be cured by complete wide-resection.

Primary breast sarcomas--report of two cases.

Two (2) primary breast sarcomas out of 110 primary breast malignancies from N.R.S. Medical College, Kolkata are being reported. Primary Breast Sarcomas are classified into five (5) broad groups with their representative features. Our two cases are classified as fibrosarcoma and malignant haemangioendothelioma and their features are documented. Because of its rarity, we are presenting this case with brief review of literature.

Hemangioendothelioma of the sphenoid bone: a case report

Hemangioendothelioma is borderline or intermediate type of vascular neoplasm. Hemangioendothelioma is rare lesion that constitutes less than 0.5% of the malignant tumors of bone. We present a case of low-grade hemagioendothelioma of the skull in a 29-yr-old woman. She had pain, diplopia and exophthalmos of the left eye. Radiographic images showed a relatively well-demarcated, expansile osteolytic lesion with irregularly thickened trabeculae and calcifications in the left greater wing of sphenoid bone. Histologically, the tumor was an infiltrative vasoformative lesion. The vessels are generally well-formed with open or compressed lumina surrounded by endothelial cells showing mild atypia. It lacked frequent mitotic figures and severe atypia. Although excessive bleeding occurred during the operation, the mass was totally resected. Postoperative radiation was not necessary. She is free of disease and well 6 months postoperatively.

Hepatic hemangioendothelioma of infancy with congestive cardiac failure--report of a case.

A case of infantile hemangioendothelioma of the liver is reported in a 18 day-old male neonate. The neonate presented with CHF. He had hepatomegaly, but lacked the third component of the classical triad i.e. cutaneous hemangiomas. The S.A.F.P levels were raised to 920 ng/ml, though rise is rare in infantile hemangioendothelioma and led to the clinical diagnosis of hepatoblastoma. A left hepatic resection was performed, the histology showed a type I pattern of the tumor. This is known to be associated with a good prognosis. The baby is well post resection for a follow up period of six months.

Hemangioendotelioma maligno primário da tiróide: caso 05/95 (RG 763.043 - Hospital Säo Paulo/EPM) / Primary malignant hemangioendothelioma of the thyroid gland: case 05/95 (RG 763.043 - Hospital Säo Paulo/EPM)

Apresentamos um caso de paciente com nódulo na glândula tiróide com 10 meses de evoluçao, de crescimento rápido e indolor. A cintilografia tiroidiana evidenciou a existência de nódulo frio. O resultado da punçao aspirativa por agulha fina (PAAF) foi suspeito (classe III). O exame de congelaçao diagnosticou nódulo benigno. No exame anatomopatológico observou-se neoplasia de origem vascular. Foi realizado a impregnaçao pela prata (reticulina) e exames imunohistoquímicos. Os antígenos pesquisados foram a vimentina, a tiroglobulina, a calcitonina, o antígeno carcinoembrionário, a enolase específica, o marcador neuroendócrino, a cromogranina, a citoqueratina AEl AE3 e o fator VIII. O diagnóstico foi definido pela imunodemonstraçao de vimentina, compatível com hemangioendotelioma maligno. O paciente foi novamente operado para complementaçao da tiroidectomia, sendo encaminhado a quimioterapia pós operatória, a qual abandonou no 3( ciclo. Encontra-se no 4( ano de pós operatório livre de doença, com PCI negativa e níveis baixos de tiroglobulina.

Tumores vasculares do ângulo pontocerebelar: relato de 2 casos e revisäo da literatura / Vascular tumors of the cerebellopontine angle: report of 2 cases and review of the literature

Os autores relatam 2 pacientes portadores de tumores de origem vascular situados na regiao do ângulo pontocerebelar. O primeiro paciente era portador de hemangioma do cavo de Meckel com extensao para o ângulo ponto cerebelar, cuja sintomatologia predominante era hipoestesia em território do nervo trigêmeo. O segundo tratava-se de um paciente portador de hemangioendotelioma localizado próximo ao meato acústico interno, cujos sintomas relacionavam-se à disfunçao dos nervos coclear e facial. Ambos foram totalmente removidos através de acesso retromastóideo e técnica microneurocirúrgica. Os tumores vasculares sao raros nesta regiao e constituem importante diagnóstico diferencial com os tumores mais comuns como o neurinoma e o meningioma. É apresentada revisao da literatura sobre o tema, com ênfase maior nos aspectos clínicos e diagnósticos.

Hemangioendotelioma ósseo: avaliaçäo de três casos / Hemangioendothelioma of bone: evaluation of three cases

O hemangioendotelioma ósseo é uma neoplasia óssea de malignidade intermediária raramente evoluindo com metástase pulmonar. Säo analisados aspectos clínicos, radiográficos e de tratamento deste tumor, sendo descritos três casos que foram submetidos a ressecçäo marginal com adjuvante ou a ressecçäo ampla, com bom resultado final e com seguimento médio de dez anos.

Primary malignant hemangioendothelioma of jejunum.

Primary malignant hemangioendothelioma is a rare tumor. We report a patient with a malignant jejunal hemangioendothelioma which had metastasized to the regional lymph nodes and the liver.