hamartoma

..Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date...

..How mTOR dysregulation after inactivation of LKB1 or TSC1/2 contributes to hamartoma development is not known...

..These findings are consistent with eccrine angiomatous hamartoma. There was no change in the lesion during the 1-year follow-up period.

..3-kinase (PI3K) and mammalian target of rapamycin complex 1 (mTORC1) is characteristic feature of hamartoma syndromes...

To describe clinical, endoscopic, radiographic, and follow-up characteristics of a series of patients in whom endobronchial hamartoma (EH) had been diagnosed.

..Any other clinically detectable primary tumor was not found. These two neoplasms were interpreted as a cholangiocarcinoma arising in BDHs. This suggested BDHs might be a risk factor of development of cholangiocarcinoma...

..We report a case of AOT in the right maxillary anterior region in a 20-year-old woman. The tumor showed some unusual and aggressive features that suggested it was a true neoplasm...

Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumor-like lesion prevalent in childhood which may produce pain and marked sweating...

..The authors herein describe the clinical, radiologic, and pathologic features of an unusual perirenal tailgut cyst with an emphasis on its histogenesis and distinction from other intraabdominal cystic lesions...

The authors report a case of hepatic mesenchymal hamartoma diagnosed prenatally with ultrasound scan and magnetic resonance imaging (MRI) and confirmed histologically postdelivery...

..The therapy of these tumors has evolved over time and it currently involves a combination of surgery, adjuvant and neoadjuvant chemotherapy and possible transplantation...

Four patients with eccrine angiomatous hamartoma are described...

Fibrous hamartoma is a key pathologic component of congenital pseudarthrosis of the tibia, a challenging and disabling bone disorder...

..Although their nature is uncertain, we propose the interim designation "mucosal Schwann cell 'hamartoma'" to avoid confusion with the neural lesions that have significant associations with inherited syndromes.

BACKGROUND: Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma consisting of dilated folliculosebaceous units invested in mesenchymal elements...

OBJECTIVE: To describe the features of retinal astrocytic hamartoma using optical coherence tomography (OCT). DESIGN: Retrospective, observational, noncomparative case series...

..This is in contrast to sebaceous trichofolliculomas which often present as depressed ostia containing terminal or vellus hairs...

The prognosis for patients with hypothalamic hamartoma has improved dramatically over the last 20 years, for 3 main reasons. First, because of improved understanding of the anatomy and pathophysiology of these varied lesions...

..sarcoma of the liver is a highly malignant and aggressive tumor that occasionally arises within mesenchymal hamartoma of the liver (MHL), a benign tumor that typically occurs in young children...

Endobronchial hamartoma is a rare, benign tumor usually associated with pneumonitis and atelectasis caused by obstruction. Lobectomy is sometimes performed even if the tumor is benign...

..The myoid hamartoma is a rare benign lesion of the breast and has an uncertain origin, possibly in the walls of the blood vessels, ..

The histogenesis of folliculosebaceous cystic hamartoma (FSCH), including the origin of the frequently associated adipocytes and other mesenchymal components, remains unclear...

Eccrine angiomatous hamartoma (EAH) is a rare condition characterized histologically by an increased number of eccrine structures and numerous vascular elements...

..CONCLUSION: Although there are pathognomonic features that characterize lipofibromatous hamartoma on MRI, the range of appearances is broad...

..These findings were most characteristic of the entity known as eccrine angiomatous hamartoma (EAH), an uncommon tumor that may present variable clinical and histological features...

To describe the computed tomographic (CT) features of Brunner's gland hamartoma with histopathologic correlation.

Muscular hamartoma is a well-demarcated tumour composed of smooth muscle and adipose tissue. We report the MRI findings of a patient with benign breast neoplasm...

Fibrolipomatous hamartoma is an uncommon congenital disorder, which is characterized by disproportionate hyperplasia of adipose tissue infiltrating along the perineurium, the epineurium and the affected nerve trajectory...

This report describes a rare case of secondary carpal tunnel syndrome due to a lipofibromatous hamartoma of the median nerve...

..From these findings a muscular hamartoma of the breast, a rare subtype of hamartoma, was diagnosed.

..resection of the affected ends from the pseudarthrosis site of both patients showed a picture compatible with hamartoma. This is the first time when histologic evidence of hamartomatous tissue involving the pseudarthrosis site is ..

..After detailed sonograms and MRI suggested the diagnosis of cystic mesenchymal hamartoma of the liver, cyst decompression was favored and consent was obtained...

Imaging findings were reported in an unusual case of endobronchial lipomatous hamartoma obstructing the left mainstem bronchus...

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...

Hepatic mesenchymal hamartoma (HMH) is a rare liver tumour in childhood. It is believed to be a benign tumour with a good prognosis if the tumour is excised. Only a few cases with local relapse or metastasis have been reported...

Fibrous hamartoma of infancy (FHI) is a fast growing soft tissue tumor that usually arises in the first 2 years of life. The histology of the lesion has been well described...

..The histological findings were compatible with chondromatous hamartoma. We recommend the endoscopic electrosurgical snaring to treat endobronchial hamartoma, especially when ..

Mesenchymal hamartoma of the liver is a rare congenital disorder of biliary tract development. During the necropsy of a late-term equine fetus, a markedly enlarged liver of more than two times normal weight was found...

Two cases of cardiac vascular hamartoma were detected in slaughtered cattle. In each case, a single nodular protrusion (ca 2.5 cm in diameter) was located in the same part of the right atrium...

..A clinical diagnosis of ovarian tumor was established before a histological diagnosis of ovarian vascular hamartoma was made. Ovarian vascular hamartoma has not been reported previously in a live animal.

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units associated with mesenchymal elements. Two cases of FSCH with typical histopathological features are reported...

Hypothalamic hamartoma is a rare congenital nonneoplastic heterotopia consisting of neurons, glial cells and fiber bundles. Clinically, most patients with hypothalamic hamartomas present with precocious puberty and/or gelastic epilepsy...

Hepatic mesenchymal hamartoma is a rare benign tumour in children...

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..The resection via rigid endoscopy with ND-YAG laser and forceps was successful, and no complications occured. The rigid endoscopy with laser-application in such case is a safe and effective procedure in endobronchial hamartochondroma...

Folliculosebaceous cystic hamartoma (FCH) is a recently recognized cutaneous hamartoma composed of follicular, sebaceous and mesenchymal components, and usually occurring on the head and neck...

BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative...

..Histology revealed the typical features of a fibrous hamartoma. Cytogenetic studies revealed a reciprocal translocation, t(2;3)(q31;q21), as the sole abnormality...

We report a six-month-old male infant with mesenchymal hamartoma of the liver which was left untreated. Repeat CT scan 1 year later showed regression and calcification of the tumor. The child is well 3 years later.

..We present here a rare case of a 7-month-old infant girl with CPP and HH who was successfully treated with depot gonadotropin-releasing hormone (GnRH) analogue is presented...

..This study aims to examine the outcomes of ten patients after orbitozygomatic (OZ) pterional surgery in cases of refractory epilepsy caused by hypothalamic hamartomas (HH)...

BACKGROUND: We review the surgical treatment of hypothalamic hamartoma causing precocious puberty...

This report describes a case of smooth muscle hamartoma of the abomasum in a 6-month-old steer humanely killed because of severe pneumonia. At necropsy, marked thickening of the abomasal wall in the area of the pylorus was found...

Mesenchymal hamartoma of the liver is an entity with a varied presentation and frequent initial delay in diagnosis...

..revealed papillary growth to extend deeply into ductular lumina, indicative of progression into early hamartoma-like neoplasia...

..Based on the gross and histologic appearance, this lesion was identified as a vascular hamartoma with arteriovenous fistula...

BACKGROUND/AIMS: Hypothalamic hamartoma (HH) is one of the most frequent causes of organic central precocious puberty (CPP). We compared the clinical presentation and the magnetic resonance images (MRI) of 19 patients with HH aged 5...

..Similar lesions have also been previously reported under the term 'acquired smooth muscle hamartoma' (ASMH). These cases are also rare and have no known consistent clinical features...

Mesenchymal hamartoma of the liver is the second most common benign liver tumor of childhood. Ultrasonography demonstrates a cystic lesion that may be very large...

..An unexpected pathological diagnosis of endobronchial lipomatous hamartoma was made...

..histologic types of soft-tissue masses can occur with tuberous sclerosis, we present a unique case of fibrous hamartoma of infancy presenting as large infiltrating cutaneous and subcutaneous masses in the abdominal wall in a 4-year-..

This article reports a case of perinatal mesenchymal hepatic hamartoma and reviews the literature on the subject. A fetus presented with polyhydramnios and a large multiloculated cystic abdominal mass at 33 weeks of gestation...

To summarize the post-operative complications of resection of hypothalamic hamartoma (HH) through transcallosal transseptal interforniceal approach.

..Histologic evaluation of the mass supported a vascular hepatic hamartoma. Vascular hamartomas are rare malformations in animals and only two cases have been reported in the dog...

Eccrine angiomatous hamartoma is a rare entity characterized histologically by the combination of proliferative eccrine and vascular elements...

Lipofibromatous hamartoma is a very rare benign peripheral nerve tumour. It is mostly encountered in the proximal extremities of young adults, involving the median nerve in the majority of cases...

To evaluate visual outcome of eyes with combined hamartoma of the retina and retinal pigment epithelium (RPE).

..Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity...

The LHRH-secreting hypothalamic hamartoma (HH), a congenital malformation consisting of a heterotopic mass of nervous tissue that contains LHRH neurosecretory neurons attached to the tuber cinereum or the floor of the third ventricle, ..

Eccrine angiomatous hamartoma (EAH) is a rare, benign condition recognized histologically by increased numbers of eccrine elements, as well as numerous vascular channels...

..contrary, good evidence now exists that all these clinical features are caused, directly or indirectly, by the hamartoma. Resection of these lesions was long regarded as too hazardous and unlikely to benefit seizure control...

To describe the imaging features of hepatic mesenchymal hamartoma with emphasis on magnetic resonance imaging (MRI) compared to histopathologic results.

Chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH) of the nasal cavity is an exceedingly rare benign lesion of the nasal cavity...

..A persistent weight gain was noted in 3 patients, which was severe in 1 (20 kg). There were no other neurologic, neuropsychologic, or neuropsychiatric side effects, which compares favorably with most surgical series...

..Here we report the effects of interstitial radiosurgery in the treatment of this generally pharmacoresistant epilepsy syndrome...

..We review the literature on ACCs in pediatric patients regarding brain metastases and previous associations with NF-1...

..form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report ..

..The signal intensity on the Magnetic Resonance Imaging (MRI) was consistent with the Hypothalamic Hamartoma (HH)...

..Alternative surgical routes and techniques have therefore been proposed, especially for broadly attached lesions and for those with a third ventricular location...

..In conclusion, radiosurgery can play a role in treating hamartomas since neurological outcome and seizure control are apparently improved...

..As with other authors, we found no potential for CNS hamartoma to proliferate. In three cases with pubertal anomalies we found CNS gliomas, which indicates the need for MRI...

..The purpose of this study is to report our experiences with endoscopic removal of these hamartomas...

..This case report presents a 29-year-old woman who was successfully rendered free of gelastic seizures after resection of a frontal cortical dysplasia, localized through MRI and SPECT imaging.[Published with video sequences]...

The human hypothalamic hamartoma (HH) is a rare, intrinsically epileptogenic lesion associated with gelastic seizures, but the underlying mechanisms remain unclear...

..METHODS: Studies were performed with 3T and 1.5T scanners. Single voxel hamartoma spectra were acquired by using short-echo-time point-resolved spectroscopy sequences (PRESS)...

PURPOSE: To present the results of transcallosal surgical resection of hypothalamic hamartoma (HH) in 26 patients with refractory epilepsy in a prospective outcome study...

..They consist of Punctate Keratoderma, Naxos Disease and Generalized Basaloid Follicular Hamartoma Syndrome...

..In this proposal, I wish to explore other mechanisms of hamartoma formation...

..are to elucidate the molecular signaling pathways that contribute to tumor development and physiology in the hamartoma syndromes resulting from loss of the tumor suppressor genes TSC1, TSC2, LKB1, and PTEN, and to identify and ..

..TSC is characterized by hamartoma formation in a wide range of tissues with the most severe clinical problems in brain and kidney...

Tuberous sclerosis complex (TSC) is a genetic disease characterized by hamartoma formations in a wide range of tissues. Mutation in either the TSC1 or TSC2 tumor suppressor gene is responsible for TSC...

Tuberous sclerosis (TSC) is an autosomal dominant hamartoma syndrome due to mutations in either TSC1 or TSC2...

..Human hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures, which are refractory to ..

..Last, the in vivo interaction in the mouse between Tsc2 null alleles and null alleles for p53, p27KIP, p21CIP, Msh2, and gelsolin will be explored. ..

..This study will elucidate the role of tumor suppressor tuberous sclerosis complex 2 (TSC2) and Rho family of small GTPases in LAM and will identify novel targets for combinational pharmaceutical intervention to treat this disease. ..

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..Finally, these in vitro and xenograft models can be used to investigate TSC tumor microenvironment and drug mechanisms of action, increasing our understanding of how these hamartomatous tumors form and respond to treatment. ..

..The results of these studies will augment the understanding of the pathogenesis of cyst formation and hypertension in ADPKD and are likely to have a significant impact on the treatment of this disease...

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..Finally it will be determined whether hyaluronan constructs that are optimal in vitro for cell migration are also optimal in vivo for cutaneous wound healing. ..

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..This study will elucidate the role of tumor suppressor tuberous sclerosis complex 2 (TSC2) and Rho family of small GTPases in LAM and will identify novel targets for combinational pharmaceutical intervention to treat this disease. ..

..Approximately one-half of these patients will have normal or near-normal renal function and one-half have mild to moderate renal insufficiency. The protocols for the Pilot Study will be conducted only at the Mayo Clinic. ..

..In summary, these studies will provide insight into the chronoblological factors that facilitate partial seizure expression and may provide new perspectives into treatments for poorly controlled partial epilepsy. ..

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