Lymphocyte-rich capillary-cavernous hemangioma of the mitral valve: a case report and review of the literature.

Valvular hemangioma incidence is extremely low. In this report, we describe a 62-year-old man who presented with mild edema of the lower limbs. An echocardiogram revealed an incidental 1.3-cm diameter mass on the anterior mitral valve leaflet for which he underwent surgical resection and mitral valve replacement. Histopathological examination showed a lymphocyte-rich capillary-cavernous hemangioma. The exuberant lymphoid stroma is unusual for hemangioma and represents an undescribed pattern of cardiac hemangioma. Including the present report, only 13 cases of mitral valve hemangioma have been reported to date. Most patients are adult. Mitral hemangioma originates in the atrial aspect of the valve and involves more commonly the anterior leaflet. The average maximum diameter of the lesion is 1.7 (S.D.=0.75) cm. Pure cavernous hemangioma is the predominant type of mitral hemangioma. Most of them are described as pedunculated or polypoid. Surgical excision appears to be curative. Recurrences have not been reported. Lymphocyte-rich cardiac hemangioma represents a peculiar type of hemangioma which should be included in the differential diagnosis of other vascular lesions.

Cavernous hemangioma of adult pancreas: A case report and literature review.

Pancreatic hemangioma is a rare type of benign vascular tumor. Low clinical suspicion and inability of current cross sectional imaging techniques to differentiate it from other pancreatic lesions, contribute to the difficulty in making the correct diagnosis. Without a definitive diagnosis, and due to concern for malignancy, in many instances, surgery is performed. We report a case of pancreas cavernous hemangioma in an 18-year-old female. The patient presented with three-month history of epigastric pain. Physical examination and routine blood tests were normal. Abdominal Computed Tomography scan revealed a 5 cm × 6 cm complex non-enhancing cystic mass in the head of pancreas. Magnetic resonance imaging, endoscopic ultrasonography (EUS) and EUS guided fine needle aspiration cytology were non-diagnostic. Because of uncontrolled symptoms, the patient underwent surgical resection. Histopathology and Immunohistochemical staining confirmed the diagnosis of cavernous hemangioma of pancreas.

Hepatocellular carcinoma with concomitant hepatic angiomyolipoma and cavernous hemangioma in one patient.

The risk of developing hepatocellular carcinoma (HCC) is strongly associated with hepatitis B virus infection. Hepatic angiomyolipoma (AML), a rare benign tumor, is composed of a heterogeneous mixture of adipose cells, smooth muscle cells and blood vessels. Here, we report the case of a 44-year-old man who developed HCC with a concomitant hepatic AML and a cavernous hemangioma, in the absence of cirrhosis. To our knowledge, based on an extensive literature search using the www.pubmed.gov website, this is the first report of an HCC case with both concomitant AML and cavernous hemangioma at the same position in the liver. The presence of the hepatitis B surface antigen was detected, but the liver function was normal. Clinical and pathological data were collected before and during the treatment. Hepatic AML was diagnosed based on the typical histological characteristics and immunohistochemical staining, which revealed, a positive staining with a melanocytic cell-specific monoclonal antibody. There was no evidence of tuberous sclerosis complex in this patient. Although the HCC was poor- to moderately-differentiated, the characteristics of the AML and the cavernous hemangioma in this patient did not match any criteria for malignancy. Hepatectomy followed by transarterial chemoembolization treatment were effective therapeutic methods for the adjacent lesions in this patient. This case is an interesting coincidence.

A rare case of male breast cavernous-type angioleiomyoma.

Cutaneous leiomyomas of the breast are extremely rare, particularly in men. Leiomyomas are categorized into three groups: angioleiomyomas, pilar leiomyomas and genital leiomyomas. Angioleiomyomas, or vascular leiomyomas, are benign tumors arising from smooth muscle cells of arterial or venous walls. We report the case of a 70-year-old man who was admitted to the surgery unit because of a painful lump in the left periareolar region. Ultrasound investigation showed a well-delimited, hyperechogenic, inhomogeneous nodular lesion. The final diagnosis was made after surgical excision and pathological evaluation of the mass. The histological features and immunohistochemical profile, characterized by positive expression of the spindle-shaped tumor cells for desmin and smooth muscle actin and by positive expression of the endothelial cells of the vascular channels for pan-endothelial markers CD34 and CD31, confirmed the diagnosis of a cavernous-type angioleiomyoma.

Orbital fibrous histiocytoma mimicking cavernous hemangioma on dynamic contrast-enhanced MRA imaging.

Orbital lesions include a broad spectrum of tumors, vascular abnormalities, and inflammatory conditions. High-resolution imaging has become an invaluable tool toward formulating an accurate diagnosis, and facilitates proper counseling regarding appropriate interventions. Imaging may guide whether partial excision to minimize damage to orbital structures, or en bloc removal to prevent potential recurrence, as seen in mesenchymal tumors, is indicated., Recently, dynamic contrast-enhanced magnetic resonance angiography (MRA) has demonstrated use in helping differentiate orbital vascular lesions. This imaging modality uses rapid MRI acquisition to provide noninvasive, dynamic flow information with high spatial resolution. However, even with modern imaging, reaching a diagnosis prior to histopathological analysis can be challenging. We present a case of orbital fibrous histiocytoma that appeared nearly identical to cavernous hemangioma on dynamic contrast-enhanced MRA.

Cavernous hemangioma arising from the diaphragm.

A 51-year-old man was referred to our hospital with an abnormal nodule in the right lung field. Computed tomography revealed a homogeneous nodule adjacent to the diaphragm, which appeared to be an extrapulmonary lesion. No hilar or mediastinal lymph node swelling was detected, and positron-emission tomography showed no significant uptake. At surgery, 2 red papillary tumors were found, originating from the right diaphragm, and tumor extirpation was performed. The pathological diagnosis was cavernous hemangioma.

There are no estrogen and progesterone receptors in cerebral cavernomas: a preliminary immunohistochemical study.

BACKGROUND: Literature consistently mentions that pregnancy and hormonal therapy probably increase the bleeding rate and seizure expression of cerebral cavernomas. Either increased hormonal activity or embryogenesis related abundant expression of some growth factors such as VEGF, bFGF, and placental growth factor during pregnancy were proposed to initiate angiogenic process and vascular proliferation in cavernomas, thereby increasing their bleeding rate and seizure expression. METHODS: To reveal whether estrogen and/or progesterone have direct effect on cerebral cavernomas, their receptor expressions were studied immunohistochemically in recently excised 12 cerebral cavernomas. RESULTS: Study showed no expression of either estrogen or progesterone receptors in cerebral cavernomas even the staining worked well in positive control tissues of infiltrative ductal carcinoma. CONCLUSIONS: Aggressive behavior of cerebral cavernomas during pregnancy is a commonly proven observation and attributed to some hormonal effects. However, this effect seems not related to effect of estrogen or progesterone on cavernoma tissue via receptor binding.

TuJ1 (class III beta-tubulin) as phenotypic marker of lymphatic and venous valves.

Lymphatic and venous valves are essential for unidirectional circulation; however, no specific marker has been described for these valves. Here, we show that TuJ1 (class III beta-tubulin) is expressed strongly in valve endothelium but not in nonvalvular endothelium lining of lymphatics. TuJ1 is also expressed in venous valves mainly at the tip of leaflets. In contrast, endothelial markers CD31, CD34, and factor 8-related antigen did not distinguish valves from vascular endothelium. TuJ1 is also expressed irregularly in the vascular endothelium of hemangiomas. The data suggest that TuJ1 may be a phenotypic marker of lymphatic and venous valves, discriminating lymphatic and venous valvular endothelial cells from nonvalve lymphatic and vascular endothelial cells.

Cavernous hemangioma of the iris in an infant.

Iris hemangioma is a rare intraocular tumor which can present with spontaneous hyphema. Juvenile xanthogranuloma, malignant melanoma with proliferation of vessels or hemorrhages in the region of cystic degeneration, and inflammatory granulomas are frequently confused with the true hemangiomas. Some authors have even questioned the very existence of iris hemangioma. Clinical case reports of iris hemangiomas with histopathologic evidence and clinical course after surgery are uncommon. We could not find any reports of iris vascular tumors confirmed using immunohistochemical staining with vascular markers. We report a case of a cavernous hemangioma of the iris in a 3-month-old infant treated surgically, and the histopathological findings and immunohistochemical analysis with vascular markers (factor VIII and CD34) are also shown.

[Immunohistochemical study on central nervous system cavernous hemangiomas].

OBJECTIVE: To explore the histological structure, angiogenesis, and proliferative activity of central nervous system cavernous hemangiomas. METHODS: 70 surgical samples of central nervous system cavernous hemangiomas and 20 normal brain vessel samples from patients of epilepsy and open craniocerebral trauma were stained immunohistochemically with CD34, a-SMA; VEGF, Flt-1; and TGFa, Ki67 respectively. A comparison analysis was made according to the expression intensity. RESULTS: CD34 and a-SMA were expressed in all the normal control brain vessel tissues in a manner of obvious and continuous staining. VEGF, Flt-1 and TGFa were not expressed obviously in the normal brain tissues. 47 and 50 out of the 70 cavernous hemangioma specimens were positively stained for CD34 and a-SMA respectively, and their expression was less continuous. 68 and 44 out of the 70 cavernous hemangioma specimens were positively stained for VEGF and Flt-1 respectively with diffuse distribution. 68 cavernous hemangioma specimens were positively stained for TGF-a. A significant difference in expression intensity was found for the above 5 factors between the normal control brain tissue and cavernous hemangiomas (all P < 0.05). No expression of Ki67 was detected in all samples. CONCLUSION: The biological characteristics of cavernous hemangiomas are mainly relevant to the immaturity of the vessel wall. A series of angiogenic factors play an important role in the development of the lesion. The proliferative activity of the cavernous hemangiomas needs to be studied further.

Endothelium in hepatic cavernous hemangiomas does not express the hyaluronan receptor for endocytosis.

The liver contains two distinct endothelial cell types: vascular and sinusoidal. Although cavernous hemangioma is the most common benign tumor of the liver, vascular or sinusoidal endothelial cell differentiation has not been described. An endocytic receptor responsible for the uptake and degradation of hyaluronan is present in the sinusoidal endothelium of the liver. The hyaluronan receptor for endocytosis (HARE) may therefore be a useful marker for sinusoidal endothelial cell differentiation. Using monoclonal antibodies specific for HARE, CD31, and factor VIII, we completed an immunohistochemical study of the endothelial cells of both hepatic cavernous hemangiomas and of nonneoplastic human liver. The anti-HARE monoclonal antibodies showed diffuse strong staining of nonneoplastic liver sinusoidal endothelium. No staining of nonsinusoidal endothelium or the endothelial lining of the hemangiomas was seen with anti-HARE. In contrast, diffuse strong staining for factor VIII and CD31 was present in nonsinusoidal endothelium and cavernous hemangioma endothelium. Neither factor VIII nor CD31 staining was present in the sinusoidal endothelium. In conclusion, the endothelium of hepatic cavernous hemangiomas demonstrates vascular but not sinusoidal differentiation based on the absence of HARE and presence of CD31 and factor VIII.

Sclerosed hemangioma and sclerosing cavernous hemangioma of the liver: a comparative clinicopathologic and immunohistochemical study with emphasis on the role of mast cells in their histogenesis.

BACKGROUND/AIMS: Sclerosed hemangiomas of the liver are rare. To date, their histopathology, immunohistochemistry, and the role of mast cells (MC) in their histogenesis have not been systematically studied. PATIENTS/METHODS: Clinical, histopathologic and immunohistochemical features of 20 sclerosed hemangiomas were compared with those of 18 sclerosing cavernous hemangiomas. The number of MC was quantified and compared in all cases, using a tryptase immunostain. RESULTS: Compared to patients with sclerosed hemangiomas, those with sclerosing hemangiomas were younger (mean age, 63 versus 71 years); had larger tumors (mean 6 +/- 4.73 versus 3 +/- 2.2 cm); presented with a mass more frequently, and epigastric pain less frequently. Sclerosing hemangiomas, but not sclerosed hemangiomas, were more frequent in males than in females. Sclerosing hemangiomas occurred much more frequently in the right lobe than sclerosed hemangiomas. Sclerosing hemangiomas had less fibrosis, hyalinization, and elastic fibers than sclerosed hemangiomas (p = 0.00004). Numerous thick-walled blood vessels were a feature of sclerosed hemangiomas but not of sclerosing hemangiomas. Collagen IV, and laminin were more uniformly positive in sclerosing hemangiomas than in sclerosed hemangiomas. Increased immunoreactivity for smooth muscle actin was present in sclerosed hemangiomas more often than in sclerosing hemangiomas. FVIII-R Ag, CD34, and CD31 were more diffusely positive in sclerosing hemangiomas than in sclerosed hemangiomas. In sclerosing hemangiomas, the mean number of tryptase-positive MC per high power field (MC/HPF) varied from 8.25 +/- 6.23 in vascular areas to 1.6 +/- 4.01 in sclerotic areas. In comparison, the mean number of MC in sclerosed hemangiomas, was 4.3 +/- 5.01 in vascular areas, and 0.86 +/- 0.58 in sclerotic areas (p = 0.0095). The number of MC was significantly correlated with vascular proliferation and inversely related to the degree of fibrosis (p < 0.0001). CONCLUSIONS: This study demonstrates certain distinct clinical and histopathologic differences between sclerosing cavernous hemangiomas and sclerosed hemangiomas of the liver. We have established the presence of MC in those tumors, and suggest possible involvement of the MC in angiogenesis, and the regression process and development of fibrosis.

Cavernous hemangioma of spermatic cord: report of a case with immunohistochemical study.

We present a case of spermatic cord cavernous hemangioma. A 32-year-old man presented with a circumscribed, painless mass in the left side of the spermatic cord. An orchiectomy of the left testicle was performed. A 3 x 3 x 2.5-cm mass was present in the spermatic cord area. Histologic examination and immunohistochemical study showed a benign vascular tumor composed of vascular spaces of varying size. Although cavernous hemangioma can occur in any location, the spermatic cord is an extremely rare site, and, to our knowledge, only a few cases have been previously reported.

Association of oligodendroglioma-like cell proliferation and angiomatous vasculature--coincidence or pathogenetically related lesions?

We report a case of an oligodendroglioma associated with a cavernous angioma. The patient, a 20-year-old man with an 8-year history of epileptic seizures, presented an acute onset of headache, nausea, and vomiting. Computerized tomographic scan and angiogram revealed multiple vascular malformations in both hemispheres including a cystic hemorraghic lesion with a perifocal edema attributing to a mass effect. Repeated puncture of the cyst failed to give improvement of elevated intracranial pressure. At surgery, the cyst and the underlying lesion were excised and found to be oligodendrocyte-rich tissue with malformed vascular tissue. Final histological examination revealed an oligodendroglioma associated with a cavernous angioma. As concurrence of oligodendroglioma and vascular malformation is rare, this case raises a broad range of differential diagnoses such as reactive oligodendroglial gliosis due to a vascular malformation, unusual vascularity of an oligodendroglioma as well as other kinds of cerebral tumors or malformations. The diagnostic difficulties as well as the pathogenetic and pathological significance of the concurrence of an oligodendroglioma and cavernous angioma are discussed.

Sex hormone receptors of hemangiomas in children.

OBJECTIVE: To investigate whether or not estrogen receptor and other sex hormone receptors are present in hemangioma tissues of children. METHODS: Fifty-two specimens of hemangiomas were taken from 52 children. The specimens were doubly stained with hematoxylin-eosin and enzyme-linking affinity immunohistochemistry. The prepared tissue slides were observed under the microscope to search for estrogen receptors (ER), progestogen receptors (PR) and androgen receptors (AR) in all the cells. RESULTS: ER, PR and AR were detected in various kinds of hemangiomas. The mean positive cell rate of each sex hormone receptor in different hemangioma tissues revealed no significant distinction in statistics (P > 0.05). CONCLUSIONS: The results suggest that hemangiomas are one of the target tissues of estrogen. Estrogen, ER, PR, and AR may play an important role in the growth and development of hemangiomas.

[An unusual case of intramuscular hemangioma]. / Un caso particolare di emangioma intramuscolare.

A case of cavernous-capillary intramuscular haemangioma is reported. The tumour was characterized histologically by a proliferation of capillaries, cavernous blood spaces, along with arterial and venous blood vessels, intermingled with striated muscle fibres and fat tissue. The complex mixture of vessels and the circumscribed margins of the tumour could support a congenital origin.

High levels of glycine and serine as a cause of the seizure symptoms of cavernous angiomas?

Cavernous angiomas are vascular malformations that cause neurodegeneration and symptoms including epileptiform seizures, headache, and motor deficits. Following neurosurgical removal of the angiomas, patients mostly recover well and become seizure-free. This study reports on the levels of certain amino acids in angiomas, obtained from 13 patients. Distinct zones of the angiomas were analyzed, from the thrombotic core, via gliotic, hemosiderin-infiltrated intermediate zones, to a periphery without macroscopic abnormalities. The neurotransmitter amino acids glutamate, aspartate, and GABA as well as phosphoethanolamine displayed decreasing levels from the periphery to the core, reflecting the gradual neuronal loss. Compared with normal brain tissue, there was a marked increase in the levels of serine (fivefold), glycine (10-fold), and ethanolamine (20-fold) in the peripheral zone of the cavernous angiomas. The results are discussed in relation to seizures and NMDA receptor activation, neuron-glia interactions, membrane phospholipids, and blood-brain barrier function.