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PubMedDate not supplied

[Hamartoma of the bile ducts. Report of a case and review of the literature].

OBJECTIVE: A clinical case report and literature review of the on the biliary-duct hamartoma is presented. BACKGROUND: The biliary hamartoma is a rare hepatic lesion described for the first time in 1918 by Von Meyenburg. It is consistent with biliary duct deformity and disorganization, forming cystic structures varying in sizes. METHODS: The patient is a 44-year-old female who had an ultrasonogram that showing disseminated hepatic lesions, which rendered an irregular shape to the viscera. An abdominal CT scan corroborated this hepatic lesion that appeared as multiple and low density in cystic form, disseminated through the entire organ. This study also showed a pelvic lesion in the left ligament of the uterus. The images were interpreted as malignant left ovaric tumor with hepatic metastases. The patient was taken to exploratory laparotomy. Liver biopsies and radical hysterectomy were performed. RESULTS: The frozen sections were reported as negative for malignancy and definitive diagnostic was biliary duct hamartoma and uterine myomatosis. CONCLUSIONS: The presence of biliary duct hamartoma causes diagnostic confusion and difficulty in the management of patients in that it appears to be liver metastases.

Adult↗
PubMed2001

[The diagnosis and treatment of hypothalamic hamartoma in children].

OBJECTIVE: To investigate the diagnosis and treatment of hypothalamic hamartoma in children. METHODS: Eighteen cases of hypothalamic hamartoma in children, including 9 boys and 9 girls, were examined with CT and MRI. Eleven cases underwent operation. Post-operation follow-up was conducted for 0.5 approximately 6 years. RESULTS: The main clinical features of hypothalamic hamartoma were precocious puberty and gelastic seizures, some combine with other kinds of seizures, mental retardation or congenital abnormalities. The effective rate of surgery was 91%; patients with simple precocious puberty were cured. CONCLUSION: Microsurgery is the first choice of treatment for hypothalamic hamartoma.

Adolescent↗
PubMed2002

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.

Female↗
PubMed2002

[Non-congenital smooth muscle hamartoma: 3 cases].

BACKGROUND: Smooth-muscle hamartoma is an uncommon, usually congenital cutaneous hyperplasia of the arrectores pilorum muscles. When it is acquired, it may be confused with Becker's nevus with a prominent smooth-muscle component. Both entities can be considered polar forms of a spectrum of dermal smooth muscle hyperplasia. We report three cases of acquired smooth-muscle hamartoma in adult patients. CASE REPORTS: Three healthy women in their fifties presented with adult-onset asymptomatic, multiple small papules grouped on circumscribed areas of the trunk. Results of biopsy specimens showed an excess of haphazardly oriented smooth-muscle bundles in the mid to lower dermis with an unremarkable overlying epidermis. Immunohistochemical studies showed two components in this proliferation: a smooth muscle cell population expressing vimentin and smooth muscle actin, and another population of CD34 positive dendritic cells, interspersed among the former. DISCUSSION: Proliferation of CD34+ dermal dendritic cells has been recently described as an additional component in the proliferation of smooth muscle cells, hair follicles and nerve fascicles that constitute congenital smooth muscle hamartomas. The results of immunohistochemical studies in our three cases extend this observation to the acquired variant of cutaneous smooth muscle hamartoma, which can be included in the expanding spectrum of diseases, recently characterized by the presence of a dermal proliferation of CD34 + cells.

Female↗
PubMed2003

[A case of hypothalamic hamartoma with refractory seizures: successful treatment with gamma knife surgery].

Hypothalamic hamartomas are associated with precocious puberty and gelastic epilepsy. The seizures are often refractory to antiepileptic medications. The treatment of hamartoma is not well established. We report a 3-year-6-month-old boy was admitted because of intractable seizures occurring several times a day. The findings on magnetic resonance imaging and his characteristic seizure led to the diagnosis of hypothalamic hamartoma. The seizures were resistant to multiple antiepileptic drugs, and persisted for about 3 years, during which his cognition deteriorated. Focal radiosurgery by a gamma knife of the hamartoma successfully controlled the seizures with no neurological complications, and his mental function improved.

Child, Preschool↗
PubMedDate not supplied

Management of endobronchial hamartoma.

Pulmonary hamartomas are uncommon benign tumors with a population incidence of 0.25%. They occur in parenchymal and endobronchial locations, the latter representing 10 to 20% of the total number of pulmonary hamartomas. Parenchymal tumors are generally small (<4 cm) and well-circumscribed and present asymptomatically as an incidental radiographic finding. Endobronchial tumors are generally broad-based lobulated nodules, which grow slowly, resulting in symptoms of airway obstruction including cough, hemoptysis, dyspnea, or obstructive pneumonia. Histologically the tumors consist of varying combinations of benign elements including cartilage, connective tissue, fat, and smooth muscle. We present a 67-year-old man who presented with persistent right lower lobe pneumonia and was found to have a right mainstem endobronchial hamartoma which was resected endoscopically by laser mechanical debridement. A review of the pathobiology of endobronchial hamartoma is presented.

Aged↗
PubMed2003

[Adenoleiomyomatous hamartoma of the lung] ].

The author described an adenoleiomyomatous hamartoma of the lung. The hamartoma was well circumscribed and measured 2.5 x 3.0 x 3.0 cm. This type of hamartoma is a rare lesion in literature described only in single cases, and without performed immunohistochemistry. In our case, the hamartoma was formed by slit or irregular glandular-like spaces lined by cuboid or columnar epithelial cells. Located around these spaces, fascicles and bundles of smooth muscle tissue with a positive antibody reaction to desmin, smooth muscle actin, and calponin, were found. Reaction with CD34 and S100 protein antibodies was negative. In the discussion, the author draws attention to disunity of terms and nomenclature of similar lesions. After three years of post-operative clinical follow-up, the patient is asymptomatic and without any signs of lung disorder.

Female↗
PubMed2003

[Chondromatous hamartoma of the lung with a unique demonstration on diagnostic imaging; report of a case].

A case of pulmonary hamartoma is reported with clinical, roentgenographical and histopathologic findings. The patient was a 53-year-old woman who had undergone right hemithyroidectomy for thyroid cancer 4 years before. An abnormal shadow, which was a non-clearly demarcated tumor, 2 cm in diameter, in the left middle lung field, was noted on her routine X-ray in February 2001. Physical examination and laboratory data revealed no significant findings, but computed tomography(CT) scans of the chest showed a gathering of small-sized high-density lesions in the nodule. She underwent left S8 segmentectomy on March 21, 2001. The pathology report on the frozen section was pulmonary hamartoma. Histopathologically, the lesion was characterized by a composition of bronchial epithelium, fat tissue and cartilage, with being diagnosed as a chondromatous hamartoma of the lung. The patient's postoperative course was uneventful, and she was discharged with no supportive therapy 14 days after the operation. To date, 14 months after the operation, the patient has been in good condition, without evidence of recurrence or distant metastasis on diagnostic imaging. This case is particularly interesting because a gathering of small-sized pulmonary hamartoma was demonstrated by imaging.

Female↗
PubMed2003

Heritable syndromes with hypothalamic hamartoma and seizures: using rare syndromes to understand more common disorders.

Manifestations of non-syndromic hypothalamic hamartomas are different from those of the syndromic hamartomas, and the syndromic ones are rare. In spite of the rarity of the syndromic hamartomas, they can be a powerful biological tool to help us understand the pathophysiology of these heritable disorders. Several disorders that include hypothalamic hamartomas are reviewed here and the current understanding of the biology of the lesions is summarized.

Abnormalities, Multiple↗
PubMed2004

A case of Brunner's gland hamartoma presenting as obscure gastrointestinal hemorrhage.

Brunner's gland hamartomas are rare tumors of duodenum, they are often discovered incidentally during esophagogastroduodenoscopy or upper gastrointestinal series. These tumors arise mainly in the duodenal bulb and can present with gastrointestinal hemorrhage and intestinal obstruction. Most of Brunner's gland hamartomas are located within the range of the standard esophagogastroduodenoscope. However, they are rarely located below the third portion of duodenum. As well known, the small intestine, including the 4th portion of duodenum, jejunum, and ileum, is relatively inaccessible with routine endoscopy. Thus, the diagnosis of Brunner's gland hamartoma in these area can be delayed up to several months after onset of symptoms. We report a case of Brunner's gland hamartoma which was located in the fourth portion of the duodenum and presented as obscure gastrointestinal hemorrhage. Radiologic, surgical, and pathologic appearances are presented.

Brunner Glands↗
PubMed2005

[Endobronchial hamartoma revealed by hemoptysis].

UNLABELLED: Hamartoma is the most frequent benign tumor of the lung. Its endo bronchial location is rare. We report two cases of endo bronchial hamartoma occurring in 2 men aged 68 and 60 years respectively. The two cases were revealed by hemoptysis. Bronchial fibroscopy showed a bud respectively in the left stump and in the lower left bronchus. Treatment consisted in a pneumonectomy and a lower lobectomy. A histological examination confirmed the diagnosis of endo bronchial hamartoma. CONCLUSION: Diagnosis of endobronchial hamartoma before surgery is difficult. Pulmonary resections are often necessary because of parenchyma lelions caused bronchial obstruction.

Adult↗
PubMed2005

[Splenic hamartoma].

The Authors present a case of Splenic Hamartoma. This is a rare unique complex vascular lesion of the spleen. This is also a recently reinterpreted lesion with some persistent confusion regarding its definition, histogenesis and classification. The patient is a young woman who following a check up with a raised erythrocyte sedimentation rate underwent abdominal sonography demonstrating an incidental slightly hypoechoic nodular splenic lesion. The pathologic study of the splenectomy specimen showed a large (10 cm) sharply demarcated mass. Histologically the lesion presented a remarkably angiomatoid lobular-nodular configuration with abundant fibrosclerotic stroma with areas of calcification. The immunohistochemical study revealed within the angiomatoid tissue different types of blood vessels: a) capillaries CD31+, CD34+, CD8-; b) structures consistent with splenic venous sinuses CD31+, CD34+/- , CD8-/+; c) small veins CD31+, CD34+, CD8-. The Authors judge this complex picture as indicative of a Splenic Hamartoma with a peculiar lobular-nodular pattern that seems to coincide with the recently described SANT: Sclerosing Angiomatoid Nodular Transformation of the spleen. In this report the Authors discuss the pathology of the lesion and the problems concerning its vasular profile. It is also emphasized the Hamartoma's great variety of morphologic patterns derived from the preponderant growth of one or another of several histologic components. This is the cause of the presence in literature of some different pathologic entities which today are fairly recognized as part of a large pathologic spectrum of the same lesion. The Authors discuss the differential diagnosis of Splenic Hamartoma with other lesions as haemangiomas and inflammatory pseudotumor.

Actins↗
PubMed2004

Mammary hamartoma--a case report.

Mammary hamartoma is a rare lesion commonly associated with Cowden syndrome. It presents clinically as a fibroadenoma. The nonspecific cytological findings makes the pre-operative diagnosis of this lesion difficult. Though hamartomas of the breast are predominantly benign, co-existent malignancies within them have been reported. These lesions also have a tendency to recur; hence a correct diagnosis and extensive sampling of hamartoma is essential. We report a case of mammary hamartoma with a brief review of literature.

Adipocytes↗
PubMed2004

Hamartoma of the breast--a case report.

A 45 year old female presented with a discrete palpable mass in the right breast. Repeated fine needle aspiration of the mass yielded only adipose tissue and was reported as lipoma - breast. Microscopic features of the resected mass were evaluated in conjunction with the clinical and gross pathologic finding and a diagnosis of hamartoma was established. The histological concept of hamartoma of the breast as described in the literature is reviewed. Here we report this rare case of mammary hamartoma with an unusual abundance of adipose tissue Repeated yield of only adipose tissue in an aspirate from a discrete palpable breast mass should suggest the possibility of a hamartoma.

Biopsy, Fine-Needle↗
PubMed2006

Respiratory epithelial adenomatoid hamartoma: a case report.

Respiratory epithelial adenomatoid (READ) hamartoma is a recently described entity characterized by abnormal glandular formations arising from the epithelium of the nasal cavity. The etiology of the lesion is unclear and may be secondary to either sinonasal inflammation or developmental error. We present a case of a 54-year-old man with a unilateral nasal mass found to be consistent with READ hamartoma upon pathologic review. Although READ hamartomas are thought to be rare, awareness of the lesion is important since it may be confused with sinonasal adenocarcinoma, leading to overly aggressive treatment. Therefore, READ hamartoma should be included in the differential diagnosis of a unilateral nasal mass.

Diagnosis, Differential↗
PubMed2006

[Retroperitoneal laparoscopic enucleation of the renal hamartoma].

OBJECTIVE: To investigate the operative technique and the clinical value of retroperitoneal laparoscopic enucleation of the renal hamartoma. METHODS: Twenty-five patients of renal hamartoma received retroperitoneal laparoscopic enucleation. The patients were placed in lateral decubitus position. All laparoscopic surgical procedures were performed retroperitoneally using 3 port-sites. The tumor was exposed and resected along the edge, and the tumor clipped out with a lithotomy forceps. The renal hamartoma was confirmed through a pathological diagnosis during the operation. The resection surface was sutured with fibrin and absorbable hemostatic gauze. RESULTS: The operations were finished successfully in all patients. The diameter of tumors ranged from 1.8 cm x 2.0 cm to 4.5 cm x 4.9 cm. The operative time was 50 - 90 (mean=70) min. The blood loss was 30 - 80 (mean=55) mL. The hospital stay was 7 - 9 (mean=8) days. CONCLUSION: Retroperitoneal laparoscopic enucleation of the renal hamartoma is safe and effective, whose advantages include minimal invasive, less blood loss, short hospital stay, and rapid convalescence.

Adult↗
PubMedDate not supplied

Hamartomas of the tuber cinereum: CT, MR, and pathologic findings.

The neuroimaging studies, clinical evaluations, and surgical and pathologic findings in five children with biopsy-proved hamartomas of the tuber cinereum were reviewed. Surgical and/or MR findings showed that patients with precocious puberty had pedunculated lesions while those with seizures had tumors that were sessile with respect to the hypothalamus. The radiologic studies included six MR examinations in four patients and CT studies in all five patients. Three children presented with precocious puberty and two with seizures, one of which was a gelastic (spasmodic or hysteric laughter) type of epilepsy. MR studies were obtained both before and after surgery in two patients, only preoperatively in a third patient, and only postoperatively in the fourth child. MR was superior to CT in displaying the exact size and anatomic location of the hamartomas in all cases. The mass was isointense with gray matter on sagittal and coronal T1-weighted images, which best displayed the relationship of the hamartoma to the third ventricle, infundibulum, and mammillary bodies. Intermediate- or T2-weighted images showed signal characteristics of the hamartoma to be isointense (one case) or hyperintense (two cases) relative to gray matter. The difference in T2 signal intensity did not correlate with any obvious differences in histopathology. CT showed attenuation isodense with gray matter, and no calcium. There was no enhancement on CT. There was no enhancement on MR in the one case in which contrast medium was administered. Preservation of the posterior pituitary bright spot was noted on all pre- and postoperative T1-weighted MR scans.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1990

[The clinical x-ray diagnosis of pulmonary hamartomas].

A clinico-roentgenologic study of 67 patients with hamartoma of the lung was carried out. Hamartoma could not be even suggested unless X-ray examination was performed but even this procedure sometimes failed to identify the disease. It was far from being each case that typical roentgenologic features of hamartoma were observed. Transthoracic needle biopsy may be helpful in identifying the nature of a roentgenologically detected tumor. Roentgenologically, hamartoma should be first of all distinguished from peripheral lung cancer. When clinico-roentgenologic and needle biopsy data are inconclusive with respect to lung cancer diagnostic thoracotomy should be performed.

Adult↗