RESUMEN
Anastomosing hemangioma is a recently described vascular neoplasm, initially identified in the male genitourinary tract. Since its first description, it has been reported at multiple anatomic sites, including rare cases in the female genital tract, most in the ovary. Herein we report the largest series to date of 12 ovarian anastomosing hemangiomas identified at our institution over a 15-yr period. The patients' age at the time of resection ranged from 50 to 76 yr (median: 62 yr), 3 patients presented with symptomatic pelvic masses, 3 tumors were identified by imaging studies, and the remaining 6 were incidentally discovered in ovaries removed for other indications. All tumors were unilateral, occurred at the ovarian hilum, and contained a vaguely lobulated architecture with sinusoidal-like vessels lined by hobnail endothelial cells with minimal to no cytologic atypia. A rim of luteinized/Leydig cells with abundant, eosinophilic cytoplasm and round, centrally placed nuclei surrounding the hemangioma was present in 9/12 tumors. Reinke crystals were observed in 3 of these 9 tumors. The volume of luteinized cells relative to the vascular proliferation ranged from 2% to 30%. All tumors with luteinized/Leydig cells also displayed numerous small eosinophilic, globular intracytoplasmic inclusions within the endothelial cells. The 3 tumors without luteinized/Leydig cells were exclusively intravascular lesions. Despite the frequent presence of luteinization/Leydig cells none of the patients experienced hormonal manifestations. Awareness of this rare benign ovarian entity is important, as its association with luteinized cells/Leydig cell hyperplasia (often exuberant) may be misinterpreted as a steroid cell tumor, Leydig-cell tumor, or as a mixed stromal-vascular tumor.
Asunto(s)
Hemangioma , Neoplasias Ováricas , Neoplasias de los Tejidos Blandos , Humanos , Masculino , Femenino , Ovario/cirugía , Ovario/patología , Células Intersticiales del Testículo/patología , Hiperplasia/patología , Células Endoteliales/patología , Hemangioma/cirugía , Hemangioma/química , Hemangioma/patología , Neoplasias Ováricas/cirugía , Neoplasias Ováricas/patología , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
A simple bone cyst (SBC) is a cystic bone lesion predominantly affecting young males. The cyst is lined by a fibrous membrane and filled with serosanguinous fluid. EWSR1/FUS-NFATC2 rearrangements were recently identified in SBC. We here report exactly the same rearrangement in 3 lesions diagnosed as vascular malformations of 2 elderly patients. In total, through Archer FusionPlex, fluorescence in situ hybridization and/or reverse transcriptase-polymerase chain reaction the EWSR1-NFATC2 rearrangement was identified in 6 of 9 SBC, 3 of 12 benign vascular tumors, and none of 5 aneurysmal bone cyst lacking USP6 fusion. Using fluorescence in situ hybridization, it was apparent that amplification of the fusion, as seen in EWSR1-NFATC2 round cell sarcomas, was absent, and that in the vascular tumors the fusion was present both in the lining cells as well as in the surrounding spindle cells. Of note, not all of the spaces in the vascular malformations were lined by endothelial cells. Aggrecan was positive in all cases but was not specific. NKX2-2 and NKX3-1 staining were negative in all cases. Thus, even though the overlap between the 2 entities is limited to the presence of few thick-walled cysts lacking endothelial lining in the benign vascular malformations, the spectrum of benign tumors containing NFATC2 fusions should be expanded and contains not only SBC in the young, but also vascular malformation/hemangioma in elderly patients.
Asunto(s)
Biomarcadores de Tumor/genética , Quistes Óseos Aneurismáticos/genética , Fusión Génica , Reordenamiento Génico , Hemangioma/genética , Factores de Transcripción NFATC/genética , Proteína EWS de Unión a ARN/genética , Adolescente , Adulto , Agrecanos/análisis , Biomarcadores de Tumor/análisis , Quistes Óseos Aneurismáticos/química , Quistes Óseos Aneurismáticos/patología , Niño , Femenino , Predisposición Genética a la Enfermedad , Hemangioma/química , Hemangioma/patología , Proteína Homeobox Nkx-2.2 , Proteínas de Homeodominio/análisis , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Masculino , Persona de Mediana Edad , Reacción en Cadena de la Polimerasa Multiplex , Proteínas Nucleares , Fenotipo , Factores de Transcripción/análisis , Proteínas de Pez Cebra/análisisRESUMEN
Hobnail hemangioma, also known as targetoid hemosiderotic hemangioma, is an uncommon vascular proliferation that clinically presents as a small solitary red to purple papule or macule, located on the limbs or trunk. Multiple lesions and atypical locations have been described. Histopathologically, it exhibits a biphasic pattern, with dilated vessels in the superficial dermis and angulated vessels in the deeper dermis, with endothelial cells that show a hobnail appearance. There is controversy about the histogenetic origin of hobnail hemangioma, although recent studies support that it is a lymphatic malformation. The investigators report the case of a 41-year-old man with an irregular lesion, red to purple in color, with a maximum diameter of 4 cm, on the scalp. The location and in particular the clinical appearance are uncommon. Immunohistochemical analysis showed negativity for WT1 and focal positivity for D2-40. Clinical-pathologic correlation acquires particular importance in the case of lesions with atypical clinical presentation.
Asunto(s)
Neoplasias de Cabeza y Cuello/patología , Hemangioma/patología , Cuero Cabelludo , Neoplasias Cutáneas/patología , Adulto , Neoplasias de Cabeza y Cuello/química , Neoplasias de Cabeza y Cuello/diagnóstico , Hemangioma/química , Hemangioma/diagnóstico , Humanos , Masculino , Glicoproteínas de Membrana/análisis , Neoplasias Cutáneas/química , Neoplasias Cutáneas/diagnóstico , Proteínas WT1/análisisRESUMEN
We report on a Spitz nevus intermingling with a hemangioma in the same biopsy from the right thigh of a 10-year-old boy. The hemangioma was made of dilated vessels in superficial areas but of narrow and angulated vessels in the deep and lateral zones. The Spitz nevus was typical, showing maturation, and no worrisome cytological features. The immunohistochemical study demonstrated expression by the vascular component of CD31, CD34, factor VIII-related antigen, and Wilms tumor 1, whereas the vessels did not express D2-40 human herpes virus 8 or glucose transporter-1. The melanocytic component expressed HMB-45 (weakly and mainly in the superficial zones), Melan-A and S-100 protein. A perivascular continuous layer of pericytes expressing smooth muscle actin was also evident.
Asunto(s)
Hemangioma/patología , Neoplasias Complejas y Mixtas/patología , Nevo de Células Epitelioides y Fusiformes/patología , Neoplasias Cutáneas/patología , Biomarcadores de Tumor/análisis , Biopsia , Niño , Hemangioma/química , Humanos , Inmunohistoquímica , Masculino , Neoplasias Complejas y Mixtas/química , Nevo de Células Epitelioides y Fusiformes/química , Neoplasias Cutáneas/química , MusloRESUMEN
BACKGROUND: Hobnail hemangioma is a small benign vascular malformation of the superficial and mid-dermis with variable clinical presentation. OBJECTIVES: To review the clinical characteristics of hobnail hemangioma in pediatric patients. METHODS: A retrospective chart review performed of all histopathologically confirmed cases of hobnail hemangioma from May 2000 to December 2014. Data on demographics, clinical characteristics, and results of immunohistochemical staining were collected. RESULTS: Four male and 2 female patients identified. Congenital lesions were reported in 3 cases. The most common anatomic location was the extremities. Treatment options included observation and surgical excision. CONCLUSIONS: Hobnail hemangioma is an uncommon benign vascular malformation. Due to its benign nature, treatment is not required. If treatment is indicated, complete surgical excision appears to be the most effective option.
Asunto(s)
Hemangioma/química , Hemangioma/diagnóstico , Neoplasias Cutáneas/química , Neoplasias Cutáneas/diagnóstico , Adolescente , Anticuerpos Monoclonales de Origen Murino/análisis , Niño , Femenino , Transportador de Glucosa de Tipo 1/análisis , Hemangioma/patología , Humanos , Masculino , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/análisis , Neoplasias Cutáneas/patología , Proteínas WT1/análisisRESUMEN
BACKGROUND: Targetoid hemosiderotic hemangioma is an uncommon, vascular, benign solitary lesion of lymphatic origin that can be misdiagnosed as other tumors including melanomas. OBJECTIVES: To evaluate the dermoscopic features of a large series of targetoid hemosiderotic hemangiomas. METHODS: Digital dermoscopic images of 35 histopathologically confirmed cases of targetoid hemosiderotic hemangiomas collected from 7 hospitals in Spain and Italy were evaluated for the presence of dermoscopic structures and patterns. RESULTS: The results of our study reveal that the presence of central red and dark lacunae and a peripheral circular reddish-violaceous homogeneous area is the most common dermoscopic pattern in targetoid hemosiderotic hemangioma (71.4%). The targetoid hemosiderotic hemangiomas of our study were correctly diagnosed in 77% of cases. CONCLUSION: Dermoscopy is helpful in improving the diagnosis of targetoid hemosiderotic hemangioma. However, attention must be paid to those cases that dermoscopically may show nontargetoid patterns, which often mimic other lesions, including melanoma.
Asunto(s)
Hemangioma/patología , Neoplasias Cutáneas/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Dermoscopía , Femenino , Hemangioma/química , Hemosiderina/análisis , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Cutáneas/química , Adulto JovenRESUMEN
Littoral cell angioma (LCA) is an extremely rare benign splenic tumor with typical histomorphologic features, which is difficult to be distinguished preoperatively from other benign or malignant splenic tumors. It is a unique vascular tumor of the spleen, with abdominal pain or as an incidental finding when undergoing physical examination. In this paper, we reported three cases of LCA treated in our hospital. Their clinical symptoms, pathological features, clinical treatment as well as the prognosis are discussed.
Asunto(s)
Hemangioma/patología , Neoplasias del Bazo/patología , Dolor Abdominal/etiología , Adulto , Biomarcadores de Tumor/análisis , Hemangioma/química , Hemangioma/complicaciones , Hemangioma/cirugía , Humanos , Inmunohistoquímica , Imagen por Resonancia Magnética , Masculino , Microscopía Electrónica , Persona de Mediana Edad , Esplenectomía , Neoplasias del Bazo/química , Neoplasias del Bazo/complicaciones , Neoplasias del Bazo/cirugíaRESUMEN
AIMS: Cells expressing markers of mast cells, macrophages and dendritic cells have previously been demonstrated within the interstitium of infantile haemangioma (IH). This study characterised these myeloid cellular subpopulations within IH. METHODS: Immunohistochemical staining was performed on proliferating and involuted IHs for the expression of Nanog, tryptase, CD163, DC-SIGN and CD45. The presence of mRNA corresponding to Nanog, tryptase α/ß-1, tryptase ß-2, CD163 and DC-SIGN was confirmed by NanoString and RT-PCR in snap-frozen IH tissues. RESULTS: Immunohistochemical staining showed expression of Nanog by interstitial phenotypical mast cells within proliferating IH, which were separate from the interstitial M2-polarised macrophages that also expressed DC-SIGN, a dendritic cell marker. These two myeloid cellular subpopulations in IH did not express the pan-haematopoietic marker, CD45. CONCLUSIONS: There are two interstitial subpopulations of myeloid cells within IH: phenotypical mast cells which also express Nanog, indicating a primitive phenotype; and M2-polarised macrophages which also express DC-SIGN.
Asunto(s)
Biomarcadores de Tumor/análisis , Hemangioma/química , Macrófagos/química , Mastocitos/química , Biomarcadores de Tumor/genética , Proliferación Celular , Niño , Regulación Neoplásica de la Expresión Génica , Hemangioma/patología , Humanos , Inmunohistoquímica , Lactante , Macrófagos/clasificación , Macrófagos/patología , Mastocitos/clasificación , Mastocitos/patología , Fenotipo , ARN Mensajero/análisis , Reacción en Cadena en Tiempo Real de la PolimerasaRESUMEN
Littoral cell angioma (LCA) is a rare vascular tumor of the spleen. It has an immunohistochemical staining pattern that is somewhat distinctive but can still be occasionally confused with other vascular and stromal proliferations in the spleen. In this study, LCA was evaluated using Ets-related gene (ERG) and Wilms tumor-1 (WT-1), relatively recently described vascular markers. In addition, other vascular lesions including normal spleen, hemangiomas, hamartoma, peliosis, and sclerosing angiomatoid nodular transformation were evaluated using these stains. In LCA, ERG stains the endothelial cells of the tumor as expected. ERG also was uniformly positive in vascular elements of other lesions except peliosis. However, in contrast to most other vascular elements, LCA was negative for WT-1 staining. This staining pattern may prove useful in diagnosing LCA and may provide insight into the derivation of the distinctive tumor.
Asunto(s)
Proteínas de Unión al ADN/análisis , Hemangioma/patología , Neoplasias del Bazo/patología , Factores de Transcripción/análisis , Proteínas WT1/análisis , Proteínas de Unión al ADN/metabolismo , Hemangioma/irrigación sanguínea , Hemangioma/química , Humanos , Inmunohistoquímica/métodos , Inmunofenotipificación/métodos , Neoplasias del Bazo/irrigación sanguínea , Neoplasias del Bazo/química , Factores de Transcripción/metabolismo , Proteínas WT1/metabolismoRESUMEN
Tufted angioma (TA) is a rare benign vascular neoplasm characterized histopathologically by the proliferation of endothelial cells arranged in lobules in the dermis and subcutaneous fat. To date, about 200 cases have been reported, most of which are of Japanese ethnicity. TA predominantly affects children and young adults, developing in 80% of patients younger than 10 years. A white 72-year-old renal transplant recipient presented with 2 asymptomatic dusky red papules on his right leg. The lesions appeared 5 years after the start of immunosuppressive treatment. Histopathologic examination showed a proliferation of poorly canalized capillary-sized vascular structures with typical "cannonball" pattern in the dermis and subcutaneous fat. Eccrine glands were also evident focally in the stroma of capillary lobules. On immunohistochemistry, endothelial cells in the vascular tufts stained positive for CD31 and CD34 but were negative for factor VIII-related antigen, human herpes virus 8, and podoplanin (clone D2-40); α-smooth muscle actin stained pericytes disposed in a single layer in capillary-sized vessels and in 2-3 or more layers in vessels of larger size, respectively. The microscopic findings were suggestive of TA. In the deep dermis, venules with smooth muscle wall and arterioles, as shown by Van Gieson staining, normally not found at that level, were present and appeared surrounded by capillary lobules. Onset of TA in adulthood is rare and may be associated with pregnancy, varicella zoster virus infection, and pharmacological immunosuppression. A case of acquired adult-onset TA associated with an arteriovenous malformation in an elderly transplanted patient is described.
Asunto(s)
Malformaciones Arteriovenosas/complicaciones , Hemangioma/etiología , Trasplante de Riñón/efectos adversos , Neoplasias Cutáneas/etiología , Anciano , Malformaciones Arteriovenosas/diagnóstico , Biomarcadores de Tumor/análisis , Biopsia , Diagnóstico Diferencial , Hemangioma/química , Hemangioma/patología , Hemangioma/cirugía , Humanos , Huésped Inmunocomprometido , Inmunohistoquímica , Inmunosupresores/efectos adversos , Masculino , Valor Predictivo de las Pruebas , Factores de Riesgo , Neoplasias Cutáneas/química , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Factores de TiempoAsunto(s)
Hemangioma/patología , Neoplasias Cutáneas/patología , Neoplasias Vasculares/patología , Vénulas/patología , Biomarcadores de Tumor/análisis , Biopsia , Niño , Diagnóstico Diferencial , Hemangioma/química , Humanos , Masculino , Valor Predictivo de las Pruebas , Neoplasias Cutáneas/química , Neoplasias Vasculares/química , Vénulas/químicaRESUMEN
Merkel cell carcinoma (MCC) is an aggressive tumor with an uncertain histogenesis typically arising on sun-damaged skin of the elderly. It has rarely been described in association with other tumors such as epithelial, melanocytic, and mesenchymal, but not vascular neoplasias. However, an exuberant vascular reaction is considered a common phenomenon in neuroendocrine neoplasms, where it can even obscure the proliferating cells and mimic a primary vascular neoplasia. A 47-year-old man was referred for the evaluation of a long-lasting reddish skin tumor located on the right buttock. After 2 punch biopsies, the patient underwent surgical excision of the lesion. Histological examination showed a dermal florid, benign vascular proliferation overlying some large deep coalescent nodules made up of monomorphous round cells with scant cytoplasm and a high mitotic activity. After the appropriate immunohistochemical stainings, a final diagnosis of hemangioma overlying a previously unrecognized MCC was rendered. Whether the angiomatous proliferation should be considered an exuberant reaction to the tumor, induced by an angiogenetic drive, or a true hemangioma is somewhat controversial. The main point is that such a neoplastiform angiomatous proliferation may represent a potential diagnostic pitfall, especially in limited specimens, and that an accurate clinicopathologic correlation is always needed. In our case, even if additional punch biopsies had been performed, the vascular proliferation would dominate the histological picture and an accurate diagnostic conclusion would probably not be reached due to the deep location of the MCC.
Asunto(s)
Carcinoma de Células de Merkel/patología , Hemangioma/patología , Neoplasias Cutáneas/patología , Biomarcadores de Tumor/análisis , Biopsia , Carcinoma de Células de Merkel/química , Carcinoma de Células de Merkel/cirugía , Errores Diagnósticos , Hemangioma/química , Hemangioma/cirugía , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Neovascularización Patológica , Valor Predictivo de las Pruebas , Neoplasias Cutáneas/química , Neoplasias Cutáneas/cirugía , Tomografía Computarizada por Rayos XRESUMEN
Papillary haemangiomas were recently defined as morphologically distinct and benign cutaneous haemangiomas showing a predominantly intravascular capillary proliferation within dilated thin-walled dermal blood vessels. We describe the case of a 45-year-old woman who presented with multiple eruptive red-bluish raised papules and nodules distributed over the skin of the chin that were related to a papillary haemangioma.
Asunto(s)
Capilares/patología , Neoplasias Faciales/patología , Hemangioma/patología , Neoplasias Primarias Múltiples , Antígenos CD34/análisis , Biomarcadores de Tumor/análisis , Biopsia , Capilares/química , Neoplasias Faciales/química , Femenino , Hemangioma/química , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Pronóstico , Factores de TiempoRESUMEN
Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described distinctive gastric mesenchymal entity with a peculiar plexiform pattern, bland spindle cells and a myxoid stroma rich in arborizing blood vessels. In this study, we report a new case of this rare gastric tumor with a subset of tumor cells derived from smooth muscle differentiation. A 32-year-old Chinese man was admitted with a gastric mass. He did not experience any discomfort, and gastroscopy showed an elevated mass in the anterior wall of the gastric antrum. Endoscopic ultrasound examination revealed a focal hypoechoic lesion protruding into the lumen. A partial gastrectomy was performed, and the patient made an uneventful recovery and remains well 3 years later. The tumor in this case depicted all the typical histopathologic and immunochemical features of gastric PAMT, except that a small subset of tumor cells was partially immunoreactive for desmin and H-caldesmon. Based on the findings of this case, we think that PAMT may contain tumor cells derived from smooth muscle differentiation, and therefore this tumor may be more than just purely myofibroblastic in nature.
Asunto(s)
Diferenciación Celular , Hemangioma/patología , Miocitos del Músculo Liso/patología , Miofibroblastos/patología , Neoplasias Gástricas/patología , Adulto , Biomarcadores de Tumor/análisis , Biopsia , Gastrectomía , Gastroscopía , Hemangioma/química , Hemangioma/cirugía , Humanos , Inmunohistoquímica , Masculino , Miocitos del Músculo Liso/química , Miofibroblastos/química , Neoplasias Gástricas/química , Neoplasias Gástricas/cirugía , Resultado del TratamientoAsunto(s)
Neoplasias Faciales/química , Hemangioma/química , Complicaciones Neoplásicas del Embarazo , Receptores de Estrógenos/análisis , Receptores de Progesterona/análisis , Neoplasias Cutáneas/química , Adulto , Neoplasias Faciales/patología , Femenino , Hemangioma/patología , Humanos , Embarazo , Neoplasias Cutáneas/patologíaRESUMEN
Hobnail hemangioma is a rare, benign vascular growth that typically presents in the third and fourth decades of life. It classically presents as a targetoid lesion with a violaceous central papule surrounded by a peripheral ecchymotic rim. Common sites of involvement include extremities and trunk. We present this case of hobnail hemangioma in a 10-year- old boy because of its rarity.
Asunto(s)
Hemangioma/diagnóstico , Hemosiderina/análisis , Trastornos de la Pigmentación/etiología , Neoplasias Cutáneas/diagnóstico , Biopsia , Niño , Eritema/etiología , Hemangioma/química , Hemangioma/complicaciones , Hemangioma/patología , Hemangioma/cirugía , Hemorragia/etiología , Hemorragia/metabolismo , Humanos , Masculino , Trastornos de la Pigmentación/metabolismo , Neoplasias Cutáneas/química , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugíaRESUMEN
Tufted angioma, first recognized in Japanese literature as "Angioblastoma of Nagakawa", is a rare benign vascular tumour with a variable clinical presentation. It commonly manifests as a macule, papule or nodule in infancy or childhood in the region of the upper trunk and neck. Here in we report two cases of this rare progressive angioma as lesions of the eyelid in adults. Tufted angioma has a classical "cannon ball" like appearance of vascular tufts on histopathology. Immunohistochemical staining with actin highlights the spindly stromal cells surrounding the capillaries. Complete physical examination and haematological work up is recommended in patients with tufted angioma to exclude rare association of port wine stain and Kasabach-Merritt syndrome with this rare entity. To the best of our knowledge, our cases illustrate the first case report of tufted angioma presenting as an eyelid lesion. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1230909536950947.
Asunto(s)
Neoplasias de los Párpados/patología , Hemangioma/patología , Neoplasias Cutáneas/patología , Adolescente , Adulto , Biomarcadores de Tumor/análisis , Biopsia , Diagnóstico Diferencial , Neoplasias de los Párpados/química , Neoplasias de los Párpados/cirugía , Femenino , Hemangioma/química , Hemangioma/cirugía , Humanos , Inmunohistoquímica , Masculino , Valor Predictivo de las Pruebas , Neoplasias Cutáneas/química , Neoplasias Cutáneas/cirugía , Resultado del TratamientoRESUMEN
Anastomosing hemangioma, a benign vascular neoplasm histologically simulating angiosarcoma, is newly recognized and has been described primarily in the genitourinary tract. We have encountered this lesion in the liver and gastrointestinal tract, where it can be diagnostically challenging, especially in core biopsy. Herein, we described 6 cases of anastomosing hemangioma of the liver and gastrointestinal tract. They occurred in 4 women and 2 men, ranging in age from 48 to 71 years. The tumors ranged from 0.2 to 6 cm (median, 3.1 cm) and were grossly well demarcated with a gray-brown spongy appearance. Microscopically, they had a loosely lobulated architecture. At higher magnification, lesions consisted of anastomosing sinusoidal capillary-sized vessels with scattered hobnail endothelial cells. Mild cytologic atypia occurred in all cases. Mitoses were absent. Vascular thrombi were seen in 4 cases (66.7%) without necrosis. One tumor (16.7%) featured prominent extramedullary hematopoiesis and 1 (16.7%) hyaline globules. Immunohistochemistry results were available for 3 cases, and the lesions stained with CD34 and/or CD31. Five cases had clinical follow-up information; there were no recurrences or metastases (range, 8 to 96 mo; mean, 41 mo), and 1 patient received no follow-up after a benign diagnosis on her colon polyp. In summary, anastomosing hemangioma of the liver and gastrointestinal tract is a rare distinctive vascular neoplasm displaying overlapping features with well-differentiated angiosarcoma. Despite small numbers and limited follow-up information in our series, evidence to date supports that the lesion is benign. Awareness of this entity is essential to avoid overdiagnosis and unnecessary aggressive treatment.