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PubMed1983

Hamartoma of CNS associated with precocious puberty.

A male infant had precocious puberty and hamartoma of the CNS. Signs of puberty appeared and progressed from 6 months of age. A computed tomographic scan disclosed an interpedunculary tumor. A craniotomy was successfully performed at 11/2 years of age, and 90% of the tumor was removed. Histologically, the tissue was identified as a hypothalamic hamartoma. Pubertal development stopped. The patient is now 4 years 9 months old and well. Review of medical literature covering a span of 47 years showed 50 cases of hamartomas in or near the hypothalamus confirmed by surgical exploration or autopsy. The male-female ratio of hamartomas with precocious puberty derived from these data is 2:1. Convulsions, mental retardation, or behavioral disorders were present in 48% of the cases; 36% had precocious puberty.

Brain Neoplasms↗
PubMed1990

Hypothalamic hamartomas and sexual precocity. Evaluation of treatment options.

We describe four male patients with hypothalamic hamartomas associated with sexual precocity. Our assessment of their management suggests that resection using current microsurgical techniques is a valid treatment option if the patient has a normal pubertal endocrine makeup, if the hamartoma is pedunculated, and if the patient is young enough to require years of parenteral medical treatment. Such surgical treatment can be curative, and subsequent growth and development can be normal (patients 1 and 2). However, if the patient is near to pubertal age (patient 3) or if neurosurgical or gonadotropin releasing hormone analogue treatment is not available, the natural history (patient 4) suggests that the only undesirable effects are accelerated growth, tall stature for age, and premature sexual development during childhood, as well as the psychosocial problems that may accompany them. Adult height may be compromised, although the two patients who did not undergo a surgical procedure and did not receive gonadotropin releasing hormone analogue therapy are above the lower limits of the normal range of adult male height. Therefore, if the hamartoma is pedunculated and cessation of pubertal development is desired, resection of the hamartoma is a reasonable therapeutic option.

Antineoplastic Agents↗
PubMed2001

Chest wall hamartoma with Wiedemann-Beckwith syndrome: clinical report and brief review of chromosome 11p15.5-related tumors.

A girl born with a left chest wall hamartoma, macroglossia, nevus flammeus of the middle forehead, and a small umbilical hernia developed left lower extremity hemihypertrophy by 1 year of age and is assumed to have Wiedemann-Beckwith syndrome. Hamartoma of the bladder and a cardiac fibrous hamartoma have been reported previously in association with Wiedemann-Beckwith syndrome. Infantile hamartomas are exceedingly rare and add to the spectrum of tumor formation in the syndrome.

Adolescent↗
PubMed1996

Myoepithelial hamartoma of the small bowel: report of a case.

Benign small bowel tumors seldom cause symptoms, due to the fluid content and distensibility of the small bowel. We herein present the case of a solitary ileal hamartoma causing melena and abdominal pain in a 24-year-old man. The diagnosis of a submucosal ileal tumor was made after performing small bowel barium studies. Surgical treatment was undertaken, and a histological examination of the excised lesion, which showed a partially ulcerated tumor surface and extended from the submucosa to the subserosa, revealed numerous cystic glands of various sizes together with bundles of proliferating smooth muscle cells. Histochemical and immunohistochemical investigations were performed for differential diagnosis, and the tumor features were consistent with a diagnosis of ileal myoepithelial hamartoma. In the literature, small intestinal myoepithelial hamartomas are quite rare and this is the first report of a myoepithelial hamartoma causing melena.

Adult↗
PubMed1978

Neuroradiologic study of hamartomas of the tuber cinereum and hypothalamus.

Five cases, four histologically proven, of hamartoma of the tuber cinereum and hypothalamus in children are reported. The ages of the patients range from 2 to 12 years. Three cases had pubertas praecox, and in all of these the hamartoma was located in the basal cistern between the chiasm and pons and had a collar button shape and size typical of hamartoma of the tuber cinereum. In the third case, a huge calcified mass in the suprasellar region was initially thought to be craniopharyngioma. The fourth case had a hamartoma within the substance of the hypothalamus and presented with hyponatremia and temporal lobe seizures.

Age Factors↗
PubMed1996

Radiological features of a symptomatic splenic hamartoma.

Symptomatic splenic hamartomas are rare in the pediatric age group, with only four previous reports in the literature. Splenic hamartoma has been reported as a solid homogeneous mass without calcification on CT and ultrasound (US), and only one previous report of the findings on MRI has been published. We report a case of a large symptomatic splenic hamartoma in a 14-year-old girl who presented with splenomegaly, pancytopenia and growth retardation. A solid mass with multiple punctate foci resembling calcifications was seen on US. The mass was heterogeneous and better demarcated on enhanced CT. Radiocolloid scintigraphy demonstrated uptake within the lesion, but less than that of normal spleen. The mass was isointense relative to normal splenic tissue on T1-weighted MRI (0.5 T) and of increased intensity with T2 weighting. At splenectomy, a red pulp hamartoma was identified, which contained nodules of hyalinization and necrosis thought to account for the punctate foci seen on US.

Adolescent↗
PubMed1985

Pubertas praecox and hypothalamic hamartoma.

Precocious puberty of cerebral origin is classified into pseudoprecocious puberty and true precocious puberty. Pseudoprecocious puberty is caused by HCG secreting tumours. True precocious puberty is caused by various hypothalamic diseases. Among them, hypothalamic hamartoma is the most common cause. Precocious puberty is caused by elevated blood pituitary gonadotropin concentration, secondary to the elevated hypothalamic LHRH secretion. The hypothalamic hamartoma is not infrequently associated with laughing (gelastic) seizures as well as convulsions. Diagnosis of a hypothalamic hamartoma is easily made by CT. Although the hypothalamic hamartoma is difficult to operate on, the value of surgery is stressed for treatment of precocious puberty. This is also confirmed by recent reports.

Child↗
PubMed1995

Giant hypothalamic hamartoma: an unusual neonatal tumor.

A case of neonatal manifestation of giant hypothalamic hamartoma is reported. It is suggested that hypothalamic hamartoma should be included in the list of neonatal intracerebral tumors. Magnetic resonance imaging appearance similar to that of normal gray matter on T1-weighted images and slightly hyperintense on T2-weighted images, without enhancement after gadolinium injection, is suggestive of the diagnosis. Hypothalamic hamartomas are congenital malformations, consisting of disorganized mature neuronal elements in proportions similar to that of normal tissue [1]. They are clinically evidenced in infants ranging from 1 to 7 years of age [1-5]. This report describes a histologically proved giant hypothalamic hamartoma diagnosed in the neonatal period. Magnetic resonance imaging (MRI) is helpful to distinguish this congenital non-evolutive malformation from more aggressive neonatal tumors.

Female↗
PubMed2005

Occult spinal dysraphism and Pacinian hamartomas.

CASES: The authors report two patients with occult spinal dysraphism who, following histological analysis, were found to harbor associated Pacinian hamartomas. One patient's hamartoma was found in conjunction with their lipomyelomeningocele and the other was combined with a presacral myxopapillary ependymoma associated with a fatty filum terminale. DISCUSSION AND CONCLUSION: We review the literature and believe this to be only the third report of Pacinian hamartomas in association with occult spinal dysraphism. Although Pacinian hamartomas are seemingly rare in conjunction with occult spinal dysraphism, mesodermal elements are often found in this disease entity.

Hamartoma↗
PubMed2000

Diencephalic neuronal hamartoma associated with congenital obstructive hydrocephalus, anophthalmia, cleft lip and palate and severe mental retardation: a possible new syndrome.

A male infant was born with severe hydrocephalus, bilateral cleft lip/palate, left anophthalmos and right microphthalmos, and an equino-varus foot deformity. Imaging studies showed enlarged lateral ventricles, apparent absence of the corpus callosum and a midline density in the third ventricular region. He had a normal male karyotype. He was severely mentally retarded and died suddenly at 7 years of age. Neuropathological examination of the brain revealed enlarged and polygyric cerebral hemispheres, due to congenital obstructive hydrocephalus, and secondary thinning of the corpus callosum. An unusually large neuronal hamartoma filled the interpeduncular fossa and third ventricle. It was continuous posteriorly with the left thalamus and so was classified as diencephalic rather than as hypothalamic. The right optic nerve merged with the hamartoma, whereas the left nerve was absent. Microscopically the hamartoma consisted of mature grey matter interspersed with narrow bands of white matter. No immature or non-neural elements were identified. This combination of diencephalic neuronal hamartoma, hydrocephalus, ocular and craniofacial abnormalities has not, to our knowledge, previously been described.

Anophthalmos↗
PubMed2005

Biliary hamartomas with delayed 99mTc-diisopropyl iminodiacetic acid clearance.

We report a 61-year-old asymptomatic man who had a hepatic lesion whose nature was undetermined, based on biochemistry, serology, ultrasonography, computed tomography, and magnetic resonance imaging studies. Biliary hamartomas were diagnosed by echo-guided needle biopsy. Functional studies of bile excretion, using 99mTc-diisopropyl iminodiacetic acid (99mTc-DISIDA) scintigraphy liver single-photon emission computed tomography (SPECT) showed the delayed transit of tracers from hepatocytes to the biliary hamartomas and the delayed emptying of tracers to the neighboring bile ducts. Therefore, biliary hamartomas should be included in the differential diagnosis of single or multiple hepatic lesions whose nature is undetermined, and a liver biopsy would be warranted for further treatment plans. We believe this is the first functional study of bile excretion in biliary hamartomas using radionucleotide studies; such studies may have prognostic implications.

Bile Acids and Salts↗
PubMed2004

"Hamartoma" of the spleen (splenoma) in children.

Hamartomas of the spleen or splenomas, are uncommon benign tumorous growths in this organ which have not been well characterized in children. We report four patients, 4 to 11 years old, who had splenomegaly and splenic "hamartomas" associated with different hematologic conditions (refractory microcytic anemia, sickle cell anemia, hereditary spherocytosis, and dyserythropoietic hemolytic anemia). All patients had total splenectomy as a primary therapeutic approach or to lessen their transfusion requirements. In only one patient was a focal splenic mass identified preoperatively with contrasted computed tomography (CT) scans and magnetic resonance imaging (MRI). None of the patients showed a mass by ultrasonography. Gross examination showed enlarged spleens (315-724 g) which on cut surface revealed a single nodule in one and multiple bulging nodules in three specimens. The nodules varied from 1.3 to 7 cm and were indistinct from the surrounding nonlymphoid splenic (i.e., red pulp) parenchyma. Histology of the nodules showed red splenic pulp with variable histiocytic proliferation, focal extramedullary hematopoiesis, lympho-plasmacytosis, fibrosis, and siderotic-calcific deposits. Intranodular small T- and B-cell lymphoid aggregates but no organized secondary follicles or periarteriolar sheaths were seen. Proliferation antigen Ki-67 (Mib-1) immunostains showed a low (< 5%) proliferation index in the nodules and surrounding tissue. Reticulin stains did not show a capsule or border between the normal spleen and the nodules. The critical histologic differential diagnosis for these lesions is with benign vascular tumors. These can be identified by their more disorderly pattern, by immunohistochemistry and by their higher proliferation index. It is our contention that these splenic nodules are not true hamartomas, as they seem to result from remote ischemic or infectious/inflammatory insults, leading to the fibro-inflammatory reaction and deposition of calcium and hemosiderin that is better designated with the descriptive term of splenoma. Review of the literature and our own experience indicates that most children with splenic hamartomas or splenomas as we prefer to call them, have an underlying hematologic disorder likely made worse by a state of hypersplenism that explains the consistent improvement in the blood values after splenectomy.

Adolescent↗
PubMed2004

Nasal chondromesenchymal hamartoma: report of a case and review of the literature.

The sinonasal region is the site of several hamartomatous lesions, the majority of which are mesenchymal, with vascular hamartomas predominating. The occurrence of hamartomas in the nasal cavity of infants and children is especially rare. Nasal chondromesenchymal hamartoma (NCMH) is a rare lesion of the intranasal sinuses generally diagnosed in the newborn period, with the eldest reported patient presenting at 16 years of age. This neoplasm is composed of mesenchymal-stromal and chondroid tissue in varying proportions. It is felt to be analogous to the mesenchymal hamartoma of the chest wall, a lesion of similar histology generally involving the ribs and chest wall of neonates. To the best of our knowledge, only 14 cases of NCMH have been reported to date. We report a case of NCMH in an 11-year-old boy.

Cartilage↗
PubMed1995

Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study).

Patients having a hypothalamic hamartoma frequently present epileptic attacks of laughter, and they later experience multiple additional seizure types, which invariably lead to a severe drug-resistant epilepsy. If this association is now well-known, relationships between the hypothalamic mass and the different types of seizures remain still mysterious. We report the case of a 16-year-old girl suffering from this peculiar epileptic picture, in whom a stereo-EEG study was performed, allowing us to record both the hamartoma, the neighboring hypothalamic structures, and other bilateral cortical areas. It showed that gelastic fits were strictly linked to ictal discharges which began and remained well localized in the hamartoma. Conversely, atonic seizures, which might result from a secondary epileptogenesis, admitted a widely extended bilateral frontal cortical origin, sparing the lesion, and slightly involving the posterior hypothalamus. Stereotactic radiosurgery of the hamartoma proved to be ineffective on both types of seizures, probably because of the too low dose of X-rays delivered (18 grays), as suggested by the absence of hypothalamic mass changes on MRI. Such data, never reported to our knowledge, seem able to contribute to a better understanding of this very peculiar epileptic syndrome, and perhaps to a better adapted therapeutic management.

Adolescent↗
PubMed1988

Olfactory hamartomas in tuberous sclerosis.

Gross or microscopic glial hamartomas were found in the anterior olfactory lobe and olfactory germinal layer of three babies, two of them newborns, with tuberous sclerosis. In two cases microscopic hamartomas were seen in the anterior olfactory lobe, and in one of them there was a prominent nodular tumor of the olfactory tract and trigone. In addition, in both of these cases there were bilateral germinal layer tumors between striatum and septum, at the junction of the obliterated olfactory recess and the frontal horn of the lateral ventricle. Only microscopic hamartomas were present in the olfactory germinal layer of the third case. Typical subependymal germinal layer tumors were also present elsewhere in all cases; however, cortical tubers were recognized in only two of them. In all three patients, the clinical presentation and death were due to cardiac rhabdomyomas. The findings suggest that olfactory hamartomas might be relatively common in tuberous sclerosis. Involvement of olfactory structures is not surprising because the lesions seem to originate in the germinal layer, a region of the brain which is prominently involved in the disease.

Brain Neoplasms↗
PubMed1988

Bilateral mandibular hamartomas and familial natal teeth.

The case of a newborn girl with two mandibular incisor natal teeth and bilateral mandibular odontogenic harmartomas is presented. The hamartomas were evident as pedunculated masses on the posterior mandibular gum pads. The natal teeth were extracted at birth, and the hamartomas were excised at 5 months of age. Microscopic investigation of the hamartoma demonstrated the presence of all odontogenic tissues with the exception of an enamel organ. In addition, there was a strong family history of natal teeth, which may suggest a hereditary basis for the development of the odontogenic hamartoma.

Female↗
PubMed1991

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.

Aged↗
PubMed1993

Nasal hamartoma: case report and review of the literature.

Nasal masses in the pediatric population present with nasal obstruction, epistaxis and chronic rhinorrhea. We report on a 6-year-old boy with long-standing nasal obstruction. A large left nasal mass was evident on physical examination and CT scan. Techniques of functional endoscopic sinus surgery were used to resect the mass, which, on pathologic examination, proved to be a hamartoma. Hamartomas are non-neoplastic malformations, or inborn errors of tissue development. They are characterized by an abnormal mixture of tissues indigenous to that area of the body. Review of the literature revealed 5 previously reported cases of nasal hamartomas, only 2 of these in children. Our case is unique in that the hamartoma arose from the ethmoid and maxillary sinuses; previously reported cases arose from the septum and vestibule. This is the first reported case treated using a functional endoscopic sinus surgery approach.

Child↗