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At least 289 records · Page 16Linked to original sources
PubMed2002

Spontaneous regression of retinal astrocytic hamartoma in a patient with tuberous sclerosis.

PURPOSE: To report the complete regression of a retinal astrocytic hamartoma in conjunction with tuberous sclerosis. DESIGN: Observational case report. METHODS: A 12-year-old boy with tuberous sclerosis, diagnosed early in life, was found to have a retinal astrocytic hamartoma in his left eye. He had bilateral optic atrophy because of long-standing increased intracranial pressure due to a cerebral giant cell astrocytoma. He was followed on a yearly basis with fundus photography and fluorescein angiography. RESULTS: At the time of initial presentation, the minimally elevated intraretinal tumor measured 2 x 1.5 mm. A year later, the same tumor measured 1.5 x 1 mm. Two years after initial presentation, the tumor totally disappeared. CONCLUSION: This patient has demonstrated that retinal astrocytic hamartoma may undergo spontaneous regression.

Astrocytes↗
PubMed2003

Exudative retinal detachment due to small noncalcified retinal astrocytic hamartoma.

PURPOSE: To report a case of exudative retinal detachment due to small noncalcified retinal astrocytic hamartoma and review pertinent literature. DESIGN: Case report and review of literature. METHODS: Clinical examination, fluorescein angiography, optical coherence tomography, and laser treatment were performed. RESULTS: Exudative macular detachment caused by a small noncalcified retinal astrocytic hamartoma confirmed by optical coherence tomography regressed completely after laser therapy. Visual acuity improved only slightly because lamellar macular thinning developed after subretinal fluid and macular exudates resolved. CONCLUSIONS: Small noncalcified, parafoveal retinal astrocytic hamartomas may cause macular retinal detachment. Optical coherence tomography may aid in the diagnosis of the tumor. Argon laser photocoagulation may induce tumor regression and resolution of exudative detachment. Final visual acuity may be limited in some cases.

Adult↗
PubMed1983

Iris hamartomas (Lisch nodules) in a case of segmental neurofibromatosis.

Iris hamartomas (Lisch nodules), previously reported in cases of peripheral neurofibromatosis, were found in a 14-year-old girl with segmental neurofibromatosis. The girl was in the 75th percentile for height (168 cm), the 90th percentile for weight (63 kg), and the 50th percentile for head circumference (55 cm). Her blood pressure was 105/70 mm Hg. Her visual acuity (20/20 and J 1), ocular motility, pupillary responses, visual fields, color vision, stereopsis, and intraocular pressure were within normal limits. Biomicroscopy showed several tan elevated nodular hamartomas on the anterior surface of the right peripheral iris but none elsewhere in the right eye and none in the left eye. Café-au-lait spots and freckling were also limited to the right side of the body. The child had no palpable neurofibromas and was in good health and of normal intelligence. There was no family history of neurofibromatosis, multiple café-au-lait spots, axillary freckling, macrocephaly, or learning disabilities. The absence of iris hamartomas has been used to distinguish segmental neurofibromatosis from the peripheral and central or acoustic forms. Thus, their presence in this case is clinically significant. The segmental form not only produces fewer complications but carries less genetic risk. In segmental neurofibromatosis, the Lisch nodules would be expected to be unilateral rather than bilateral, ipsilateral to the side of the cutaneous involvement, and more frequently associated with contiguous cutaneous lesions.

Adolescent↗
PubMed1993

Changing clinical picture of mammary hamartoma.

During a 6-year period, we identified 12 patients (age range: 16 to 72 years) with a histologic or mammographic diagnosis of mammary hamartoma. The lesion was found in 9 of 441 open breast biopsy specimens (2%) and was identified radiographically in 5 of 8,122 mammographic examinations (less than 1%). Two groups of patients were identified. Three patients under 30 years of age underwent the excision of small palpable lesions found on pathologic examination to be mammary hamartomas (group I). In nine patients over age 30, masses were identified or confirmed on mammography (group II). Five lesions showed the classic mammographic appearance of a mammary hamartoma (a circumscribed tumor of mixed soft tissue and fatty density), and the other four were indeterminate. Presentation in these older women who had a relatively high incidence of atypical mammographic findings mandates that biopsy be performed.

Adolescent↗
PubMed1996

Tracheal hamartoma: report of a child with a neck mass.

Tracheal hamartoma represents an oddity in children. Only one case was found in the English literature as a distal tracheal intraluminal lesion causing obstructive symptoms. All other reported cases were in older patients, who were presumed to have asthma or chronic obstructive pulmonary disease. The authors report on a 21-month-old girl who presented with an anterior neck mass fixed to the trachea. Imaging studies showed a lesion (2.5 x 2.3 x 1.7 cm) anterior to the right lobe of the thyroid, extending to the trachea posteriorly and down to the thoracic inlet inferiorly. The mass had a mixture of soft tissue densities with a focus of calcification. Results of thyroid studies were normal, and there was no adenopathy. Neck exploration showed a white, firm, lobulated mass fixed to the trachea, which was resected completely. The postoperative course was unremarkable. Histological studies showed a mixture of mature cartilage, fat, fibroconnective tissue, and spindle cells with myxoid degeneration, consistent with tracheal hamartoma. Based on a literature search, this is the first reported case of extraluminal tracheal hamartoma presenting as a neck mass in a pediatric patient.

Female↗
PubMed1994

Fibrous hamartoma of infancy in the genital region: findings in 15 cases.

Fibrous hamartoma of infancy is a benign myofibroblastic proliferation that typically occurs in the axillary or shoulder region of male infants. We describe 15 cases of this condition, which involved the inguinal region in 5, scrotum in 5, spermatic cord in 1, perineum in 1, labium majus in 1, the suprapubic region in 1 and the pubic area in 1. Patient median and mean ages were 10 and 6.7 months, respectively (range 2 to 24). No case was reported to be congenital. Median and mean tumor size was 3 cm. (range 0.5 to 6). The microscopic features were identical to those seen in fibrous hamartoma of infancy occurring in more typical sites and consisted of 3 components: 1) fascicles of myofibroblasts, 2) disorganized mature adipocytes and 3) small rounded primitive mesenchymal cells. Immunohistochemically, the myofibroblastic component expressed muscle specific actin and vimentin, and the primitive component expressed vimentin only. There was no evidence of increased cellular proliferation in the primitive cell component using proliferating cell nuclear antigen antibodies. Of the 15 lesions 1 recurred locally and 14 were apparently cured by local excision. Awareness of this presentation of fibrous hamartoma of infancy may avert misdiagnosis of more aggressive lesions, especially infantile fibromatosis or rhabdomyosarcoma.

Actins↗
PubMed1978

The concept of hamartomas: its relevance to the pathogenesis of odontogenic lesions.

The term hamartoma should be applied only to tumorlike lesions which arise during development of the organ or tissue in question and which are apparently not neoplastic; it should not be used instead of benign tumor. A hamartoma is composed on an abnormal mixture of tissues native to the part, with an excess of one or more of these tissues. The relevance of the concept of hamartomas in the pathogenesis of odontogenic lesions is discussed briefly.

Hamartoma↗
PubMed1998

Hamartoma of mature cardiac myocytes.

The clinical and pathological findings of three patients with hamartomas of mature cardiac myocytes resembling localized hypertrophic cardiomyopathy are presented. Hypertrophic cardiomyopathy is manifest by a poorly demarcated area of cardiac hypertrophy, microscopically demonstrating myofiber disarray and intramural coronary thickening. Localized, nonencapsulated masses of hypertrophied cardiac myocytes in locations other than the left ventricle or ventricular septum have not been reported. The clinical and pathological data of three patients with localized hamartomas were retrospectively retrieved. The patients were 9, 22, and 28 years old, respectively; none had a known family history of heart disease or cardiomyopathy. Two patients had cardiac arrhythmias: one patient died suddenly, and one patient had the Wolff-Parkinson-White syndrome. The third patient was asymptomatic. Two patients treated surgically had single masses in the right atrium and right ventricle, respectively. The patient who died suddenly had multiple discrete masses throughout the atrial and ventricular myocardium, including the left ventricular free wall. None of the three patients had septal asymmetry suggestive of hypertrophic cardiomyopathy. Histologically, there were discrete but unencapsulated nodules of marked myocyte hypertrophy with disorganization, focal scarring, and thickened intramural arteries. There was no myocyte vacuolization suggestive of cardiac rhabdomyoma. Ultrastructurally, the myocytes showed abundant and disorganized myofilaments and normal intercellular junctions. Hamartoma of mature cardiac myocytes is a previously undescribed cardiac tumor that shares some features of hypertrophic cardiomyopathy and rhabdomyoma, but is currently best considered a separate entity.

Adult↗
PubMed2001

Rhabdomyomatous mesenchymal hamartoma of the eyelid: report of a case and literature review.

PURPOSE: To report a rare case of rhabdomyomatous mesenchymal hamartoma and to compare its features with those cases previously reported. DESIGN: Interventional case report and literature review. INTERVENTION: Complete ophthalmologic and systemic examinations followed by excisional biopsy and histopathologic examination. MAIN OUTCOME MEASURES: Clinical examination features and histopathologic findings. RESULTS: A 6-month-old Latino male presented with a congenital, elevated, smooth, flesh-colored right lower eyelid lesion. An ipsilateral right limbal dermoid and an upper eyelid coloboma were also present. Excisional biopsy of the eyelid lesion revealed randomly oriented mature striated muscle tissue with associated adipose tissue, blood vessels, pilosebaceous units, and peripheral nerves, findings consistent with rhabdomyomatous mesenchymal hamartoma. Of the 24 reported cases (including the current case), eight had associated congenital anomalies. CONCLUSIONS: Although rhabdomyomatous mesenchymal hamartomas are rare and benign, they may be associated with other congenital anomalies and anomaly syndromes. As a result, we recommend systemic evaluation of patients diagnosed with this entity.

Biopsy↗
PubMed2002

Glandular hamartoma of the larynx.

Glandular hamartoma of the larynx is an extremely rare lesion, and the number of well-documented and acceptable cases is limited. Presenting symptoms may include changes in voice, eating and activity levels, and respiratory complaints. We report on a 14-month-old infant with this rare clinical entity. Direct laryngoscopy revealed a well-mucosalized, encapsulated, firm, 0.5 cm wide and 2.5 cm long lesion that originated from the right aryepiglottic fold and reached into the nasopharynx. The mass was completely excised endoscopically. Histopathological examination revealed a hamartoma, which was composed of glandular elements, mixed with mesodermal tissues. After endoscopic removal of the hamartoma, the child was relieved of the obstruction.

Biopsy, Needle↗
PubMed1999

Laryngeal hamartoma: surgical management.

A newborn female infant with severe inspiratory stridor was found to have a laryngeal non-encapsulated hamartoma in the supraglottic area, medial to the hyoid bone and extending into the petiole of the epiglottis. Histologic examination revealed an extremely uncommon glandular hamartoma. Surgical management in the newborn period consisted of conservative endoscopic excision combined with open laryngofissure. The hamartoma was removed in the newborn period to avoid tracheostomy, because decanulation after tracheostomy in infants can be difficult.

Endoscopy↗
PubMed2006

[Combined hamartoma of the retina and retinal pigment epithelium. Four case studies].

INTRODUCTION: Combined hamartoma of the retina and retinal pigment epithelium is a rare condition, characterized by a proliferation of the retinal pigmentary epithelium and retinal gliosis leading to a disorganization of the retina and papilla. This study aimed to demonstrate the advantages of early diagnosis and regular monitoring. OBSERVATIONS: We report a series of four children followed between 2001 and 2004 with combined hamartoma of the retina and retinal pigment epithelium, with age of diagnosis ranging from 3 months to 8 years. The main reason for consultation was reduction of vision. The clinical examination objectified the existence of a slightly grayish peripapillary formation with tortuous retinal vessels. This condition was confirmed by angiography with fluorescein in three cases and by optical coherence tomography (OCT) in one case. Progression showed the persistence of low vision in all four cases and the appearance of a neovascular membrane in one case. DISCUSSION: Combined hamartoma of the retina and retinal pigment epithelium is probably a congenital tumor whose pathogenesis has not yet been elucidated. The diagnosis is clinical and the patient can be thoroughly examined by retinal angiography and optical coherence tomography (OCT). In this disorder, it is important to eliminate retinoblastoma and malignant melanoma of the choroid, showing the advantage of radiological exploration. Progression is stationary; nevertheless a reduction in visual acuity can be related to an epiretinal membrane or a neovascular membrane. CONCLUSION: Knowledge of the clinical aspect is essential to differentiate this condition from the malignant retinal processes. This tumor can progress in spite of its benign character. Regular follow-up is essential and can improve the visual prognosis.

Child↗
PubMed1996

Folliculosebaceous cystic hamartoma: a clinical pathologic study.

BACKGROUND: Folliculosebaceous cystic hamartoma (FCH) is a recently recognized cutaneous hamartoma comprising follicular, sebaceous, and mesenchymal elements. Only nine cases have been previously reported. OBJECTIVE: The aim of this study was to characterize further the clinical, histologic, and immunohistologic features of FCH. METHODS: The clinical, histologic, and immunohistologic features of eight cases of FCH were evaluated and the literature reviewed. RESULTS: All lesions presented as exophytic papules or nodules on the head, most commonly the central part of the face. Age at onset ranged from 4 to 47 years. The size ranged from 0.5 x 0.4 cm to 1.4 x 1.1 cm. Clinical diagnoses were nevus, neurofibroma, and adnexal tumor. None of the patients had associated Muir-Torre syndrome. The diagnostic microscopic features of infundibular cystic structures with attached sebaceous glands and characteristic stroma were present in all lesions. Many of the stromal spindle cells were CD34 positive. CONCLUSION: FCH is a recently recognized hamartoma of follicular, sebaceous, and mesenchymal elements. Although reported only rarely, its incidence is likely much higher.

Adolescent↗
PubMed2002

[Sebaceous hamartoma of Jadasshon: trichoblastoma mimicking basal cell carcinoma?].

The sebaceous hamartoma of Jadassohn is a congenital skin malformation usually appeared at birth or in early childhood. Many authors support the notion that early complete surgical excision is necessary to prevent the development of malignant neoplasms (especially basal cell carcinoma) after puberty. On the contrary, others in recent large series suggest that it is an histological misdiagnosis: the basal cell-carcinoma is in fact a trichoblastoma, basaloïd neoplasm, but non-malignant one. We have also asked anatomopathologist for proofreading slide previously described as basal cell carcinoma to research unknown trichoblastoma. During the period, 1982 to 1999, 80 sebaceous hamartoma of Jadassohn were excised and 19 basal cell carcinoma were found. The basal cell carcinoma diagnosis has been confirmed in two cases only. The others were pigmented trichoblastomas. So, trichoblastoma is the most common basaloïd tumor developed in sebaceous of hamartoma Jadassohn. Nevertheless, we should recommend surgical excision during the childhood because of best elasticity of tissue, especially on the scalp where it's mostly localized.

Adolescent↗
PubMed1998

Muscular hamartoma of the breast. Case report and review of the literature.

Muscular hamartoma (MH) is a rare tumor-like lesion of the breast, and only 22 cases have been reported in the literature. We report an additional case of MH of the breast, giving a new immunocytochemistry-based insight into the histogenesis of the smooth muscle component. The expression of CD34 antigen in smooth muscle cells suggested that MH originates from stromal cells via leiomyomatous metaplastic changes. This hypothesis was supported by the evidence that stromal cells in the normal parenchyma nearby the hamartoma were positive for CD34 antigen and, focally, also positive for smooth muscle markers (alpha-smooth muscle actin and desmin). A complete list of differential diagnoses and a review of the literature on the topic are provided. Although the use of the term "muscular hamartoma" could be considered inappropriate because of the absence of smooth muscle tissue in the normal breast, the authors underline that this designation can be easily accepted if the histogenesis of the heterologous component is regarded as a result of a stromal cell metaplastic change.

Breast Neoplasms↗
PubMed1995

Splenic hamartoma: a case report.

A case of splenic hamartoma is presented that was incidentally discovered in a 55-year-old male during a work-up to determine the aetiology of hypertension. Diagnostic imaging procedures were suggestive of a splenic hamartoma. Histologically, this was found to be of the pulposal type with haemosiderin and calcification foci and many eosinophil leukocytes. The patient had an uneventful recovery. A review of the pertinent references is included, together with a brief discussion about the occurrence of splenic hamartoma in the context of tuberous sclerosis.

Hamartoma↗
PubMed2004

Respiratory epithelial adenomatoid hamartoma of the maxillary sinus presenting as a periapical radiolucency: a case report and review of the literature.

We report a case of respiratory epithelial adenomatoid hamartoma of the left maxillary sinus that initially presented as a periapical radiolucency involving the left maxillary first molar. Respiratory epithelial adenomatoid hamartoma is a rare lesion that occurs in the nasal cavity, paranasal sinuses, and nasopharynx. Most cases are found in the nasal cavity associated with the posterior nasal septum. Involvement of the maxillary sinus is very unusual, and only one other report of a respiratory epithelial adenomatoid hamartoma involving the maxillary antrum was found in a search of the literature. The current case is additionally unique as it was initially detected in the course of a dental examination. Awareness of this lesion is important because inverted schneiderian papilloma and adenocarcinoma may be included in the histopathological differential diagnosis. Conservative surgical removal is curative and recurrence has not been reported.

Adenocarcinoma↗
PubMed1993

Hamartoma of the tongue.

A female infant nine months of age presented with a bilobed swelling at the base of her tongue. Histological examination showed it to be a hamartoma of the tongue. Lingual hamartomas are rare. There are fifteen case reports of lingual hamartoma in the world literature. Our case is presented and the previous case reports are reviewed.

Female↗