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PubMed1999

Congenital smooth muscle hamartoma of the conjunctival fornix.

PURPOSE: Congenital smooth muscle hamartomas are benign tumors composed of proliferating smooth muscle cells. They are usually seen as abnormal patches of skin. Ocular involvement of congenital smooth muscle hamartomas is unusual, with rare reports of patients with external eyelid involvement or proptosis resulting from orbital tumors. We describe a patient with a congenital smooth muscle hamartoma that involved the tarsal conjunctival fornix. METHODS: Review of the patient's medical records, including the results of ophthalmologic, radiologic, and histologic examinations. RESULTS: A healthy 2-year-old boy was initially seen with a conjunctival mass. He had a discrete, gray, cystic-appearing lesion in the inferior fornix of the left eye. A magnetic resonance imaging study revealed no signs of extension of the lesion into the orbit. The lesion was surgically excised. Histologic sections showed large bundles of smooth muscle with a fibrotic background and interdigitating fat, consistent with a diagnosis of a congenital smooth muscle hamartoma. CONCLUSION: To our knowledge, this is the first report of a patient with a congenital smooth muscle hamartoma arising from the conjunctival fornix. It presumably originated from either the smooth muscle of the vascular endothelium or from the capsulopapebral muscle. Congenital smooth muscle hamartoma should be considered in the differential diagnosis of cystic-appearing conjunctival fornix lesions.

Child, Preschool↗
PubMed1980

Clinical spectrum of pulmonary hamartomas.

Pulmonary hamartomas are tumors that occur in the parenchyma or the bronchi. Through the lesions themselves are benign, they may be associated with malignant neoplasms or they may cause obstruction of the bronchi because of their location. Pulmonary hamartomas were resected in 29 patients at the Ochsner Medical Institutions during a 30-year period. Of these patients, 6 had symptoms related to concomitant malignancy and 3 had symptoms resulting from obstruction of the bronchi by the hamartoma. Surgical treatment ranged from enucleation to pneumonectomy. One patient died in the hospital of extensive bronchogenic carcinoma. In 2 others, carcinoma of the lung developed within twelve months after resection of a hamartoma. In this group of patients, 20% had concomitant or subsequent pulmonary lesions. Ten percent of the hamartomas were located in the bronchi, resulting in bronchial obstruction. A surgical approach is indicated for lesions that appear to be pulmonary hamartomas on roentgenograms.

Adolescent↗
PubMed1999

Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature.

Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the right lobe that was overlooked initially during a left lateral segmentectomy at 18 months of age for a mesenchymal hamartoma. Malignant mesenchymoma is a rare and aggressive tumor. The origin of this tumor is not well understood. There has been no direct support to the hypothesis that malignant mesenchymoma may be the malignant counterpart of mesenchymal hamartoma. The authors provide clinical and histopathologic evidence in our case that suggests the possibility of malignant mesenchymoma arising from a mesenchymal hamartoma. This case emphasizes the need for complete removal of mesenchymal hamartoma and the need for long-term follow-up to detect multifocal lesion or malignant transformation.

Antineoplastic Agents↗
PubMed2002

Microsurgical treatment for hypothalamic hamartoma in children with precocious puberty.

BACKGROUND: We review the surgical treatment of hypothalamic hamartoma causing precocious puberty. METHODS: Six children (three girls and three boys) with precocious puberty secondary to hypothalamic hamartoma were recruited for our study. The mean age of the patients was 30 months old (range 13 months to 5 years), and the mean age of the onset of puberty was 7.3 months. All patients were treated by microsurgery. RESULTS: All patients had higher then normal stature, body weight, bone growth, and serum levels of sexual hormones. The boys presented with mature external genitalia, pubic hair, frequent erection, and acne, while the girls presented with growth of breasts and menarche. Magnetic resonance image (MRI) revealed an isointense mass below the tuber cinereum extending into the supersellar and interpeduncular cistern, ranging from 4 to 12 mm in diameter, consistent with pedunculate hamartoma. The hamartoma was removed completely via a right pterional approach. The symptoms and signs of precocious puberty resolved completely, and sexual hormone levels decreased to the pre-pubertal range in all six patients without any postoperative complications. CONCLUSION: We report a series of six children with hypothalamic hamartoma-induced precocious puberty who underwent microsurgical treatment. All of them recovered completely to their age-appropriate state. Microsurgery is a good choice of treatment for pedunculate hypothalamic hamartoma.

Brain Neoplasms↗
PubMed1997

A rare case of hamartoma in the lateral ventricle: case report.

BACKGROUND: Intraventricular manifestation of a hamartoma is an unusual clinical entity. CASE DESCRIPTION: A 12-year-old girl was admitted with complaints of dizziness and head heaviness. Computed tomography and T1-weighted magnetic resonance imaging (MRI) demonstrated an isodensity or isointensity mass lesion with cystic component in the right lateral ventricle, which showed relatively high signal intensity on T2-weighted MRI and lacked contrast enhancement. Histopathologic examination on the surgically removed specimens using glial fibrillary acidic protein, Klüver-Barrera, Bodian stainings, bromouridine labeling index, and MIB-1 immunohistochemical staining confirmed that the tumor had glial and neuronal components and low proliferative potentiality. CONCLUSION: This case is the first report of intraventricular hamartoma, although some reports on intracranial hamartoma located in areas other than the hypothalamus have been published. The radiologic findings, including relatively high signal intensity on T2-weighted MRI and absence of contrast enhancement, may be suggestive of the diagnosis of intraventricular hamartoma; however, the specific stainings for glial and neuronal cells and proliferative evaluation are essential to the precise diagnosis of hamartoma. Clinical manifestation of hamartoma in the ventricular system, though rare, should be kept in mind.

Brain Diseases↗
PubMed2002

Rhabdomyomatous mesenchymal hamartoma: an unusual dermal entity with a report of two cases and a review of the literature.

BACKGROUND: Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital lesion of the dermis and soft tissues consisting of a disordered and varied collection of mature adipose tissue, skeletal muscle, adnexal elements and nerve bundles. This entity exists under various names including striated muscle hamartoma, congenital midline hamartoma, and hamartoma of cutaneous adnexa and mesenchyme. Several published cases report the occurrence of RMH within the setting of other uncommon congenital abnormalities. METHODS: We report the clinical and pathologic features of two cases of rhabdomyomatous mesenchymal hamartoma. Patient 1 is a 71-year-old man who presented for removal of a nodule located on his temple that had been traumatized during a recent haircut. Patient 2 is a 4-month-old infant with amnion rupture sequence and rare craniofacial abnormalities including facial clefts, microphthalmia, bilateral colobomas, and a mobile fingerlike projection above the left medial canthus. RESULTS: Histological examination in both cases showed a deep dermal and subcutaneous fat collection of disorganized skeletal muscle fibers, adipose and neural tissue, and adnexal structures. Characteristically, the skeletal muscle approximated folliculosebaceous structures in a haphazard manner. CONCLUSIONS: RMH is a rare benign condition of the deep dermis and subcutaneous fat with only 22 cases existing in the English literature. To our knowledge, this is the first report of an elderly man presenting with RMH, presumptively present since birth. There was no evidence of cellular or malignant degeneration. While the etiology of RMH is unknown, possible explanations include aberrancy in the embryonic migration of mesodermally derived tissues or a genetic defect predisposing to the formation of hamartomas.

Abnormalities, Multiple↗
PubMed2003

[Respiratory epitheloid adenomatoid hamartoma of the nasal cavity--case report and review of the literature].

BACKGROUND: Hamartomas are tumour-like malformations, resulting from excessive proliferation of otherwise normal local tissue components. Hamartomas of the nasal cavity and paranasal sinuses are relatively rare, especially respiratory epithelial adenomatoid hamartomas. This benign lesion is characterized by a glandular proliferation originating from the surface of the respiratory epithelium. In the German speaking literature this entity has not been described before. PATIENT AND RESULTS: We report the case of a 51-year-old white female, presenting with a respiratory epithelial adenomatoid hamartoma of her left nasal cavity. The patient suffered from nasal obstruction. The CT scan showed a soft tissue like lesion obstructing the patient's left choana. After local resection of the tumour, which originated from the superior turbinate through functional endoscopic sinus surgery the patient's symptoms were gone. Follow-up after six months showed no evidence of recurrence. CONCLUSION: The histopathological differential diagnosis of respiratory epithelial adenomatoid hamartomas includes inverted papilloma and adenocarcinoma. Therefore the histological recognition of these hamartomas impacts on therapy. Misinterpretation as a true neoplasm may result in unnecessarily radical surgery. Local surgical resection is the treatment of choice for this lesion.

Adenocarcinoma↗
PubMed1982

Pulmonary hamartoma.

From 1973 to 1980, 9 pulmonary chondromatous hamartomas of the lung were resected at our Clinic. One of these hamartomas was a rare endobronchial chondroma. To our knowledge, 91 cases of endobronchial hamartomas have been described in the previous literature. Although a similar origin of intrapulmonary and endobronchial hamartomas is postulated, there is a difference in their clinical features. The endobronchial type of hamartoma often causes respiratory symptoms of obstructing the bronchus with consequent destruction of the distal lung, whereas patients with intra-pulmonary hamartomas are symptom-free.

Adult↗
PubMed1996

Case report: splenic hamartoma with hematologic disorders.

Hamartomas of the spleen are rare benign tumors usually detected as asymptomatic, incidental findings at laparotomy or autopsy. By review of the literature, there are only 16 well-documented cases of symptomatic splenic hamartomas associated with hematologic disorders. The authors report on an additional case of splenic hamartoma in a 34-year-old man with thrombocytopenia. Findings by ultrasonography and computed tomography suggested multiple confluent splenic lesions. Because of progressively increasing thrombocytopenia, elective splenectomy was performed. Final pathology confirmed diagnosis of multiple hamartomas of the red pulp. Platelet count returned to normal within 1 month postoperatively. Differential diagnosis, diagnostic procedures, and pathologic findings of splenic hamartomas will be discussed. Hamartomas should be kept in mind in the differential diagnosis of splenic tumors. Splenectomy is indicated in cases where malignancy cannot be excluded and in cases of associated hematologic disorders.

Adult↗
PubMed2006

Spindle cell predominant trichodiscoma: a fibrofolliculoma/trichodiscoma variant considered formerly to be a neurofollicular hamartoma: a clinicopathological and immunohistochemical analysis of 17 cases.

Seventeen solitary nasal tumors that fulfilled all diagnostic criteria of so-called neurofollicular hamartoma, apart from distinct S100-positivity, were compared histopathologically and immunohistochemically with seven typical trichodiscomas from a similar clinical setting. Both the S100-negative neurofollicular hamartoma-like tumors and the trichodiscomas expressed an identical CD13-positive/CD34-positive fibrocytic immunophenotype without co-expression of neural/perineural (S100, neurofilament, epithelial membrane antigen), myogenic (desmin, calponin, muscle-specific actin, and alpha-smooth muscle actin), or melanocytic (S100, HMB45, NKI/C3, MelanA) epitopes. Histopathologically, there was striking morphologic overlap between trichodiscoma and S100-negative neurofollicular hamartoma-like tumor, apart from a highly characteristic fascicularly organized cellular fibrocytic stroma in the latter. We conclude that fibrofolliculoma/trichodiscoma and neurofollicular hamartoma-like tumor are morphologic variants of a single hamartomatous entity in which neurofollicular hamartoma-like tumor occupies the cellular pole of the morphologic spectrum. The entity formerly known as neurofollicular hamartoma appears to be nothing but a particularly cellular trichodiscoma with a distinctively organized stroma composed of CD34-positive fibrocytes. We therefore propose the new term spindle cell predominant trichodiscoma (SCPT) for this particular variant of the morphologic fibrofolliculoma/trichodiscoma spectrum.

Adult↗
PubMedDate not supplied

Infiltrating ductal carcinoma and ductal carcinoma in situ associated with mammary hamartoma.

Mammary hamartoma is a rare nonmalignant lesion. Only 11 cases of carcinoma associated with hamartoma have been previously described in the literature. We describe a case of infiltrating ductal carcinoma (IDC) and ductal carcinoma in situ (DCIS) associated with hamartoma in a 35-year-old woman. Mammography showed the features of a typical hamartoma with suspicious microcalcifications arising in it. The patient underwent a radical modified mastectomy. It is likely that hamartoma is a coincidental finding. The identification of suspicious microcalcifications in a typical mammographic image of a hamartoma should prompt continued examination to exclude an underlying tumor.

Adult↗
PubMedDate not supplied

Reactive alveolar epithelium in chondroid hamartoma of the lung.

OBJECTIVE: To determine the morphologic characteristics of the nonciliated epithelium found in chondroid hamartoma of the lung. STUDY DESIGN: The morphologic characteristics and immunohistochemical reaction for surfactant protein A of the nonciliated epithelium in chondroid hamartoma of the lung was studied by immunohistochemistry. Alveolar epithelium in normal lung tissue and lung tissue surrounding primary lung cancer or metastatic lung lesions was used as a control. RESULTS: In all cases, the nonciliated epithelium in chondroid hamartoma showed the morphologic criteria of hyperplastic alveolar type II cells and a very strong positive surfactant protein A reaction in the cytoplasm when compared with alveolar epithelium of the normal lung. Similar hyperplastic type II cells were also found in the alveolar lung around metastatic or primary lung tumors. CONCLUSION: These findings may indicate that the nonciliated cells found in chondroid hamartoma of the lung are hyperplastic type II cells. This suggests that the alveolar epithelium found in chondroid hamartoma of the lung is a secondary reaction around the hamartoma and not a primary component of the lesion.

Adipose Tissue↗
PubMed1998

Symptomatic splenic hamartoma: case report and literature review.

An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, approximately 140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.

Adult↗
PubMed1995

The radiologic and pathologic spectrum of biliary hamartomas.

OBJECTIVE: The purpose of our study was to correlate the spectrum of radiologic and pathologic findings in a series of patients with biliary hamartomas and known extrahepatic malignancy. MATERIALS AND METHODS: Biliary hamartomas were diagnosed in 18 patients with a primary malignant lesion who had liver biopsy for evaluation of possible metastatic disease. Prebiopsy imaging studies included CT in 16 patients and sonography in 11. Imaging studies were reviewed retrospectively and correlated with findings at surgery and on pathologic examination. The hamartomas were classified histologically by the degree of cystic dilatation of bile ducts within the lesion. RESULTS: Radiologically, biliary hamartomas presented a spectrum of findings including one or two circumscribed lesions (5-10 mm in diameter) in four patients; multiple (about five) lesions (approximately 5 mm each) in one patient; innumerable tiny, nearly uniform (2-5 mm) lesions in two patients; and innumerable lesions of varying size (2-15 mm) in three patients. Among the patients with innumerable lesions, the nodules were either uniformly or nonuniformly distributed throughout the liver. In all cases, the lesions were hypodense on contrast-enhanced CT scans and hypoechoic on sonograms. In eight patients, the lesions were not visible by imaging but biopsies were done at surgery when single or multiple tiny nodules were noted on the liver surface. The diagnosis was made by either wedge or core-needle biopsy; fine-needle aspirations were nondiagnostic. Pathologic examination revealed single or multiple hamartomas of varying sizes ranging from solid to largely cystic lesions; the degree of cystic dilatation did not correlate with imaging findings. Visibility on imaging correlated with larger lesion size; small surface lesions were usually occult. CONCLUSION: Biliary hamartomas cause single or multiple nonspecific hepatic lesions that may mimic metastases. This diagnosis should be considered in patients with a primary malignant tumor when single or multiple small hepatic lesions are seen, regardless of uniformity of size or distribution.

Adult↗
PubMed1999

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma.

OBJECT: Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship. METHODS: The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the "parahypothalamic type," in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the "intrahypothalamic type," in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle. Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone-releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed. This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty. CONCLUSIONS: Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.

Adolescent↗
PubMed2002

Placental transmogrification of the lung is a histologic pattern frequently associated with pulmonary fibrochondromatous hamartoma.

CONTEXT: Placental transmogrification of the lung is a term introduced to describe a peculiar histologic pattern characterized by formation of placental villuslike structures in the lung parenchyma. It has been reported to occur in association with bullous emphysema and lipomatosis. OBJECTIVES: To study the relationship between placental transmogrification and pulmonary hamartomas. DESIGN AND METHODS: Reports of 38 cases of pulmonary hamartomas during 18 years (1982-1999) were reviewed. All histologic slides of these cases were examined for the presence of villuslike papillary projections and placenta-like structures. Hamartomas with prominent papillary projections or placenta-like structures were further investigated to assess the histogenesis and proliferation of epithelial and stromal cells. Immunohistochemical analysis was performed on paraffin-embedded tissue using monoclonal antibodies against Ki-67 and thyroid transcription factor 1 (TTF-1) and polyclonal antibodies against c-Kit antigen (a stem cell factor receptor/mast cell growth factor receptor) in conjunction with Leder stain for naphthol-ASD-chloroacetate esterase. RESULTS: Placental transmogrification was identified in 6 of 38 cases of pulmonary fibrochondromatous hamartomas. The histologic change consisted of an abundant myxoid or edematous fibroadipose stroma with a respiratory epithelial lining, resulting in papillary projections that resembled immature placental villi. Epithelium lining the papillary projections was positive for TTF-1 (70%-90%) and Ki-67 (3%-5%). In contrast, stromal cells were negative for TTF-1 with only rare cells immunoreactive for Ki-67. A number of stromal spindle cells and occasional cells in epithelium were c-Kit immunoreactive; however, concurrent Leder stain demonstrated that these c-Kit-positive cells were mast cells and not stem cells. CONCLUSIONS: Placental transmogrification is frequently associated with pulmonary fibrochondromatous hamartomas and may be induced by or associated with a proliferation of lining epithelial components in the hamartomas. The significance of numerous mast cells within stroma of placental transmogrification is unclear and their possible role in inducing stromal proliferation needs to be further evaluated.

Aged↗
PubMed1999

[Diagnosis and treatment of pulmonary hamartoma].

OBJECTIVE: To summarize the characteristics of pathogenesis, image, treatment and prognosis of pulmonary hamartoma. METHODS: 39 cases with hamartoma confirmed by pathology received surgical operation during 1971-1998 period. Their clinical data including symptoms, disease courses, image characteristics and surgery types were investigated. The prognostic results of 31 cases were collected by a follow-up study. RESULTS: The average age of morbidity was 44 years old and the mean disease course of hamartoma was 13 months. 21 cases without symptoms were determinated by chance and 18 cases with some extent of cough, expectoration, fever, chest distress, and short of breath. The hamartomas of 21 cases were in right lung, 18 in the left. Image characteristics presented as popcom-shaped tumor with clear border and without satellite focus. 31 cases underwent enucleation, 4 wedge-shaped resection, 3 lobectomy and 1 pneumonectomy. No recurrence or malignant change was demonstrated by follow-up study. CONCLUSIONS: Pulmonary hamartoma is a kind of neoplasms with satisfactory prognosis. Intraluminal hamartoma should be differentiated from hilar lung cancer. Localized resection should be performed for the treatment of the disease.

Adolescent↗
PubMed2003

From hypothalamic hamartoma to cortex: what can be learnt from depth recordings and stimulation?

Patients having a hypothalamic hamartoma (HH) frequently present gelastic or dacrystic seizures, and they often later experience multiple additional seizure types which lead to a severe epileptic encephalopathy. There is now increasing evidence that the HH itself plays a crucial role in this syndrome, but the relationships between the lesion and the different types of seizures remain a questionable issue. Stereotactic intracerebral EEG recordings were performed in 5 patients suffering from a medically intractable epilepsy associated with a HH. The hamartoma was investigated in all cases, and various cortical areas were also evaluated in 4 of the 5 patients. The epileptic discharges arose and remained confined within the hamartoma in 3 of the 4 patients in whom laughing and crying episodes were recorded. In addition, interictal spikes were recorded from the hamartoma in 4 of the 5 patients, whereas the stimulation of the HH could reproduce gelastic or dacrystic episodes in 3. The three patients in whom other types of seizure were recorded showed that the latter were associated with cortical ictal discharges not affecting the HH. Ictal onset appeared either bifrontal, right fronto-central and lateral temporal, or bifrontal with a right side predominance. The cingulate gyrus was involved in all these 3 cases, and the lateralization of the ictal discharges was always ipsilateral to the predominating side of the hamartoma. Interestingly, these seizure types were sometimes immediately preceded by the laughing or crying attacks, as if ictal discharges within the hamartoma triggered those which seemed to originate in the cortex. Therefore, if these findings confirm the intrinsic epileptogenicity of HH, they also demonstrate that epileptic seizures associated with HH can exhibit different types of electroclinical patterns. We propose a speculative pathophysiology in which the mamillo-thalamo-cingulate tract would serve as a relay of HH discharges towards the cortex, the excitability of which would then progressively increase, first leading to cortical interictal epileptiform abnormalities and then to seizures of cortical origin. Whether this proposal of secondary epileptogenesis is valid or not remains a major issue, since it could provide arguments on the moment to discuss surgery.

Adolescent↗