Contrast-enhanced ultrasound defines vascularization pattern of hamartomatous colonic polyps in Peutz-Jeghers syndrome.

The hamartomatous polyps in Peutz-Jeghers syndrome may have malignant potential. To differentiate between hamartomatous and adenomas polyps, vascular characterization can be assessed using noninvasive procedures, such as contrast-enhanced ultrasound (CEUS). The neo-angiogenic characteristics of colorectal adenomas and carcinomas are expressed as an anarchic vascular pattern observed on CEUS. Using CEUS in a patient with Peutz-Jeghers syndrome, we describe for the first time the vascularization of a hamartomatous colonic polyp that exhibits a hierarchy branching pattern.

Contrast-Enhanced Ultrasound Defines Vascularization Pattern of Hamartomatous Colonic Polyps in Peutz-Jeghers Syndrome

The hamartomatous polyps in Peutz-Jeghers syndrome may have malignant potential. To differentiate between hamartomatous and adenomas polyps, vascular characterization can be assessed using noninvasive procedures, such as contrast-enhanced ultrasound (CEUS). The neo-angiogenic characteristics of colorectal adenomas and carcinomas are expressed as an anarchic vascular pattern observed on CEUS. Using CEUS in a patient with Peutz-Jeghers syndrome, we describe for the first time the vascularization of a hamartomatous colonic polyp that exhibits a hierarchy branching pattern.

Dynamic enhancement patterns of solitary pulmonary nodules at multi-detector row CT and correlation with vascular endothelial growth factor and microvessel density.

BACKGROUND AND OBJECTIVE: Differential diagnosis of malignant solitary pulmonary nodules (SPNs) from benign ones is difficult based on imaging manifestations. This study was to assess dynamic enhancement patterns of SPNs detected with multi-detector row computed tomography (MDCT), correlate SPN manifestations of MDCT to the expression of vascular endothelial growth factor (VEGF) and microvessel density (MVD), thus to explore the potential value of MDCT imaging in the diagnosis of SPNs. METHODS: Fifty pathologically and one clinically confirmed patients with SPNs (diameter

Fibrous hamartoma of infancy manifesting as multiple nodules with hypertrichosis.

Fibrous hamartoma of infancy is a rare benign tumor, which usually occurs within the first year of life. Most cases present as a solitary, painless, skin-colored subcutaneous nodule; multiple lesions are rarely reported. There have also been a few cases which have showed overlying skin changes, including hypertrichosis and alterations in pigmentation. We report a rare case of fibrous hamartoma of infancy in a 6-month-old female who presented as multiple nodules with overlying hypertrichosis on the left buttock. To the best of our knowledge, there have been only two reported cases in the dermatological published work that showed these unusual findings simultaneously.

Colour Doppler ultrasound patterns and clinical follow-up of incidentally found hypoechoic, vascular tumours of the spleen: evidence for a benign tumour.

Between January 1990 and January 2005, incidental hypoechoic, vascular tumours of the spleen were identified in 13 patients using B-mode and colour Doppler ultrasound (CDS). All lesions found were well demarcated, intrasplenically located, and ranged in size between 1 cm and 4 cm. The increased vascular pattern on CDS was confirmed in 9 of the 13 cases by contrast enhanced ultrasound (CES), while two patients showed reduced vascularity on CES. In 10 patients, lesions were confirmed by contrast enhanced CT. Histological examination was performed in three patients with the diagnosis of capillary haemangioma (n = 2) and hamartoma (n = 1). In the remaining cases, ultrasound follow-up was performed (range 4 months to 13 years) and demonstrated no evidence of tumour growth in all but one patient. During a 4 year follow-up, one lesion increased in size from 1.0 cm to 1.5 cm and in the same patient an additional 0.5 cm sized hypoechoic increased vascular lesion was also found. In the spleen a hypoechoic lesion with an increased vascular pattern incidentally found by ultrasound most likely indicates a benign tumour with capillary haemangioma/hamartoma as the most likely diagnosis. However, it should be emphasised that in all cases a careful ultrasound follow-up is warranted.

Basket pattern blood flow signals discovered in a case of splenic hamartoma by power Doppler ultrasonography.

We present the gray-scale ultrasonography (GSUS), power Doppler ultrasonography (PDUS), abdominal computed tomography (CT), and magnetic resonance imaging (MRI) findings for a case of splenic hamartoma in a 27-year-old man, showing a phi 50 mm homogeneous, iso- and hypo-echoic splenic mass with evidence of a small plural cystic lesion. This splenic hamartoma showed increased vascularity on power Doppler sonograms. PDUS showed multiple circular blood flow signals inside the mass (i.e. a basket pattern), which was consistent with the small plural cystic lesion shown by GSUS. Spectral analysis also confirmed arterial and venous flow. CT scans showed that the mass had low-density relative to the normal spleen and MRI showed that the mass was isodense, relative to the normal spleen. Therefore, CT and MRI are not useful for the diagnosis of splenic hamartoma. Ultrasonography can be used to diagnose splenic hamartoma without administration of a contrast material and therefore is an indispensable method for the diagnosis of splenic hamartoma.

Unusual vascular hamartoma of the hard palate. A case report.

Hamartomas are tumour-like malformations usually present since birth or which may develop during puberty. Hamartomas are related to anatomical development errors. Hamartomas are rarely found in the head and neck district. The case of an unusual vascular hamartoma localised in the hard palate of a 50-year-old female with no significant medical or family history is reported. Surgical treatment was performed by means of an excisional biopsy.

[Hamartoma oligemicus]. / Hamartome oligemicus.

BACKGROUND: Naevus oligemicus is a rare affection caused by selective vasoconstriction of the deeper vessels ("thermal" skin blood flow), with respect of the superficial vascular plexus ("nutrient" blood flow). We report the fourth case. CASE REPORT: A 45 year-old man presented erythematocyanotic lesions with sensation of coolness on the abdomen and the thighs. These lesions had first been noted by the patient 3 years earlier. Physical examination and laboratory investigations were normal. Histology of a lesion revealed a non-specific superficial dermatitis. Result of skin surface thermometry of both areas showed that the surface temperature of the involved skin was up to 2 degrees C lower than the surrounding control skin. DISCUSSION: We don't know why there is a selective vasoconstriction of the deeper vessels and not of the superficial vascular plexus. Naevus oligemicus belongs to the pharmacological naevus group because of the absence of any histologic vascular abnormality. The functional abnormality has not been identified. This could be explained either by increased sympathetic vasoconstrictor tone in the autonomic sympathetic vasoconstrictor nerves supplying the deeper thermoregulatory vasculature in the affected skin, or by increased sensitivity of these vessels to vasoconstrictor catecholamines.

Mesenchymal hamartoma of liver. A regional ischemic lesion of a sequestered lobe.

OBJECTIVE: To evaluate histologic and gross features of mesenchymal hamartoma of liver and similar lesions in relation to determining likely pathogenesis. DESIGN: Case series of patients presenting to a tertiary care hospital over 16 years. PATIENTS: Three children with mesenchymal hamartoma ranging from newborn to 11 months of age and one 12-year-old girl with torsion of an accessory lobe of liver. MAIN OUTCOME MEASURES: Similarity of gross vascular and segmented anomalies as well as apparent consequent histologic features of mesenchymal hamartoma to those of torsion of an accessory lobe of liver indicate that they are pathogenetically related. RESULTS: Dissecting microscopic examination revealed a single vascular supply in one case and remote thrombi in two cases of mesenchymal hamartoma evaluated. The histologic features of mesenchymal hamartoma (hypocellular central zone and hypercellular periphery) were duplicated in the lobe of liver with torsion. CONCLUSIONS: Mesenchymal hamartoma represents a lesion with an anomalous solitary vascular supply that may evolve into its specific pattern with stromal cysts as a result of early ischemic changes.

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and delineation of two new entities.

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.

Splenic hamartoma and capillary hemangioma are distinct entities: immunohistochemical analysis of CD8 expression by endothelial cells.

The histologic and immunohistologic features of two morphologically similar splenic tumors, a capillary hemangioma and a splenic hamartoma, are reported. The hemangioma was composed predominantly of small vascular channels lined by endothelium expressing factor VIII-related antigen and lacking T-subset antigen (CD8). In contrast, the splenic hamartoma was predominantly a spindle cell lesion with numerous vascular channels coursing through the tumor; these contained splenic-type endothelium expressing both CD8 and factor VIII-related antigen. Our results justify the concept that the splenic hamartoma is a tumor of splenic origin or a true hamartoma and is distinct from the splenic capillary hemangioma.

Eccrine-pilar angiomatous nevus.

A clinical and histological study was made of a congenital lesion on the knee of a 3-year-old boy showing a peculiar combination of eccrine, pilar and angiomatous structures with the characteristics of organoid nevus. A discussion is made of different questions related to eccrine hamartomas with respect to the clinical picture, the association of other hamartomatous components, the alterations that these induce in other cutaneous structures and their histogenesis.

Fibrous hamartoma of infancy: an ultrastructural study.

The fine structures of three fibrous hamartomas of infancy were studied. All three components of these lesions were examined. The principal cells in the fibrous and myxoid areas were fibroblasts. Cells with stellate cytoplasmic projections were more prominent in myxoid areas. Myofibroblasts were abundant in two cases and not in the other. The adipose tissue component was formed by lipocytes and occasional preadipose fibroblasts. Blood vessels were more numerous in myxoid areas. Larger vessels were accompanied by smaller vessels and by clusters of cells with primitive junctions, suggesting early blood vessel formation. Some small vessels were surrounded by concentric layers of mesenchymal cells, as if attempting to form media. Electron microscopy seems to confirm the hamartomatous nature of fibrous hamartomas of infancy. The lesion appears to recapitulate the formation of blood vessels and fat, as seen in fetal tissues. Cellular myxoid areas showed prominent vasoformative proliferation, which decreased in the adipose tissue and was least prominent in the dense fibrous component. The latter may represent the end stage of the fibrous proliferation.

[Visceral arteriography of hepatic, pancreatic and intraperitoneal tumours during childhood (author's transl)]. / Die viszerale Arteriographie bei Leber-, Penkreas- und intraperitonealen Tumoren im Kindesalter.

The value of visceral arteriography is discussed in relation to four hepatoblastomas, two vascular hamartomas of the liver, one inactive islet cell tumour of the pancreas and two malignant intraperitoneal tumours. Although non-invasive methods, such as computer tomography, sonography and scintigraphy are valuable, angiography is an important part of the pre-operative diagnosis of visceral tumours during childhood.