Hepatic mesenchymal hamartoma

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. We reviewed 134 patients with space-occupying liver lesions, from January 1990 to December 1999, and four patients with HMH were found. Abdominal mass and/or distension were the common initial presentations. Three of our four cases were diagnosed in the first year of life. The liver function tests and tumour markers were non-specific. Case 1 underwent tumour enucleation and another three cases underwent tumour excision. One patient died. No tumour recurrence was noted in the surviving patients.     

Long-term follow-up of a multifocal hepatic mesenchymal hamartoma producing a-fetoprotein

We here present a 6-month-old girl with cystic hepatic mesenchymal hamartoma and elevated a-fetoprotein (aFP). Following hepatectomy of the left lobe and partial right lobectomy, decline of the serum aFP was observed. The child has been well for 20 years and it is one among a few cases with such a long observational period.     

Multicystic hepatic mesenchymal hamartoma of childhood

A case of massive mesenchymal hamartoma of the liver is described in a 13-month-old infant. A complex multicystic mass was demonstrated by ultrasound and computed tomography (CT). The appearances suggested the preoperative diagnosis.     

Skin hamartoma on the hand of a newborn infant

Skin hamartoma is an extremely rare disease on the hand in newborn infants. Reported herein is the case of a newborn infant who presented with a skin hamartoma on the hand. The patient was a girl born at 37 weeks of gestational age. The mass was seen on her proximal left thumb at birth. The mass had a spherical diameter of 4 cm and was pedunculated. One the day after birth, the stalk on the mass was ligated in the neonatal intensive care unit. The mass was diagnosed as skin hamartoma on histopathology. At 1 year of age, the child had good hand function, and no recurrence of the neoplasia was evident.     

Congenital cystic adenomatoid pulmonary hamartoma in a newborn infant

Case report of a newborn infant with acute respiratory distress. Roentgenologic signs of patches of increased density with scattered round and oval areas of radiolucency suggested the diagnosis of congenital cystic adenomatoid pulmonary hamartoma. Diaphragmatic hernia had to be excluded by a gastrografin^®-meal gastrointestinal series. During surgery a right lower lobectomy was done. The diagnosis was proved by gross and microscopic examinations of the affected parenchyma of the lung.     

Suction lipectomy of a congenital lipomatous hamartoma

A 4-month-old baby with a diffuse lipomatous hamartoma of the lower limb was operated upon by repeated suction lipectomies. The result 2 years later was satisfactory. Correspondence to: A. Franchella     

Hepatic mesenchymal hamartoma in a preterm newborn: demonstration by low-dose multidetector CT

Primary liver tumours are very rare in the neonatal period. Differential diagnoses include haemangioendothelioma, malignant hepatoblastoma and mesenchymal hamartoma. Due to non-specific clinical symptoms and indecisive imaging findings, correct diagnosis may be difficult to establish. We report a female preterm newborn who was delivered at 33 weeks of gestation and in whom ultrasonography (US) revealed a large cystic intraabdominal tumour of unknown origin. For further evaluation, contrast-enhanced multidetector computed tomography (CT) was performed on the 4th day of life using a low-dose protocol (80 kVp, 50 mAs, collimation 0.75 mm, total effective dose 3.6 mSv). Based on CT findings, diagnosis of an intrahepatic mesenchymal hamartoma was made and confirmed by tumour resection and histopathological examination. In conclusion, multidetector CT (MDCT) using a low-dose protocol can be exceptionally used to establish diagnosis of a mesenchymal hamartoma in a preterm newborn.     

Mesenchymal hamartoma of the liver in children

Mesenchymal hamartoma of the liver is regarded as a rare developmental abnormality that occurs most frequently in children. In the last 12 years we found 115 reported cases, and we present 2 additional ones. The literature, reveals, however, that microhamartomas are much more common in adults. The patients have a history of rapid abdominal enlargement and are otherwise relatively asymptomatic. Ultrasound and computed tomography are the most useful diagnostic tests. Most tumors can be removed without sacrificing normal liver parenchyma.     

Fibrous hamartoma of infancy in the scrotum

Fibrous hamartomas of infancy and childhood are rare benign tumors. The clinical and morphological features of these lesions can be different according to the age at onset. Although the most common localizations are the shoulders, axillas, and upper arms, these lesions may develop in unusual sites such as the scrotum. The authors report a 13-month-old child with a fibrous hamartoma in the scrotum, which was excised. At 1-year follow-up there was no sign of local recurrence or distant disease. The clinical and histological features of the disease are described in order to facilitate correct diagnosis and avoid inadequate therapy. Offprint requests to: F. Ferro     

Mesenchymal hamartoma of the liver

Eight children ranging in age from 1 day to 6 years with mesenchymal hamartoma of the liver are reported. The diagnosis was established by the characteristic ultrasound finding of a mass of mixed cystic and solid consistency. The treatment of choice is operative resection, which was accomplished with low postoperative morbidity and no mortality. By reducing excessive vascularity, angiographic embolisation facilitated resection in one patient.     

Central precocious puberty due to hypothalamic hamartoma in a 7-month-old infant girl

Hypothalamic hamartomas (HH) are rare congenital lesions of the tuber cinereum presenting with the classic triad of gelastic epilepsy, central precocious puberty (CPP) and developmental delay. In light of the important and diverse consequences of precocious puberty for affected children and their families, a correct diagnosis without delay is imperative. 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.     

新生儿先天性肝血管瘤伴动静脉瘘导致心功能不全1例北大核心CSCD

患儿女,3日龄,因生后气促3 d入院。患儿为胎龄36+2周早产儿。产前胎儿超声提示胎儿肝血管发育异常,生后即出现呼吸急促,需要呼吸支持。胸部X线检查提示心影增大,心脏彩超提示右心房和右心室增大,肝脏超声、腹部增强CT明确诊断为肝血管瘤伴动静脉瘘。19日龄时在全身麻醉下行肝动脉结扎术,术后心功能好转出院。基因检测显示ACVRL1基因变异,该变异来自患儿母亲。该文主要介绍1例由肝血管瘤伴肝动静脉瘘导致新生儿心功能不全病例,以及对该疾病的多学科诊疗。     

Mesenchymal hamartoma of the chest wall

Mesenchymal hamartoma of the chest wall is an unusual lesion remarkable for its occurrence in early infancy and alarming clinical presentation, which often suggests malignancy. Despite its aggressive appearance and propensity to attain massive size, the lesion behaves biologically in a benign fashion, and may be treated conservatively, provided that there is no respiratory compromise secondary to mass effect. The case of an incompletely resected mesenchymal hamartoma that has behaved in a benign fashion after more than 4 years of follow-up is discussed here. We present this case to provide insight and raise clinicians’ awareness of the behavior of an extremely rare tumor.     

Torsed pedunculated hepatic hamartoma

We report a 9-year-old boy with a 6-h history of acute abdominal pain due to torsion of a pedunculated hepatic mesenchymal hamartoma. The lesion was seen, on US and CT, to connect to the liver through a pedicle. Mesenchymal hepatic hamartomas are unusual tumours that may be pedunculated, and this is a unique case complicated by torsion. The radiological and pathological findings, differential diagnosis, and clinical course are discussed.     

Laparoscopic extirpation of splenic hamartoma

We presented a case of splenic hamartoma. A 12-year-old girl has had an abdominal pain since 9 years of age. Contrast-enhanced computed tomography scan at a local hospital revealed heterogeneous enhancement of a mass which is 5 cm in diameter, located near the surface of the spleen, diagnosed as hemangioma. She had an investigation with Levovist-enhanced ultrasonography and superparamagnetic iron oxide-enhanced magnetic resonance imaging on T2-weighted gradient-echo imaging, which showed a hypervascular echoic mass and a decrease of signal intensity. The imaging diagnosis was splenic hamartoma. She underwent laparoscopic splenectomy to prevent the spontaneous rupture. The splenic artery at the pancreas body was exposed, and doubly ligated by vessel clip and furthermore ligated by 3-0 silk. At the splenic hilum, the splenic artery and vein were dissected with the Endo GIA vascular linear cutting stapler. The spleen was captured into the Endocatch II retrieval bag and removed from a transverse lower abdominal (suprapubic) incision extended up 7 cm in length through a port site. In pathological findings, the tumor was compatible with hamartoma. Splenic hamartomas in pediatric patients have been described extremely rare. In clinical symptoms, spontaneously ruptured splenic hamartoma has been rarely reported.     

True precocious puberty of a girl with hamartoma of the CNS successfully treated by operation

A girl with precocious puberty due to a hypothalamic hamartoma is presented. At the age of 0.41 years vaginal bleeding was documented and signs of puberty were noted: PHIII, BII according to Tanner. The bone age was 1.3 years, and height velocity rose from the 50th to 90th percentile. Plasma concentrations of LH (5.85 mU/ml), FSH (3.29 mU/ml), growth hormone (30 ng/ml), and oestradiol (90 pg/ml) were elevated. The results of a neurological examination including an EEC recording as well as a skull roentgenogram, were unremarkable. The visual evoked potentials were grossly abnormal. A native and contrast CT scan visualized a tumour close to the suprasellar cisterna reaching the chiasma opticum.At the age of 1.2 years the tumours was removed. Histologically the tissue was identified as a hamartoma. Immediately after the operation vaginal bleeding ceased, pubertal development regressed, bone age did not advance any further, the visual evoked potentials normalized and the contrast CT did not show any tumour mass. The levels of LH, FSH, growth hormone and oestradiol 4 months post operation were decreased as follow: LH: 1.14 mU/ml, FSH: 0.70 mU/ml, GH: 15.1 ng/ml, oestradiol: 10 pg/ml. However, there was an increase of FSH (3 mU/ml) 1 year after the operation. No secondary sexual characters reappeared.     

Malignant potential of mesenchymal hamartoma: an unrecognised risk

A patient who had undergone an incomplete resection of a mesenchymal hamartoma of the liver at 9 months of age presented 8 years later with an undifferentiated embryonal sarcoma. Offprint requests to: L. Spitz     

Intraluminal hamartoma of the ileum in a child: a rare cause of intestinal occlusion

A case of small-intestinal obstruction in a 12-year-old boy caused by an intraluminal hamartoma of the ileum is reported. The rarity of the lesion, its location, symptomatology, histology, and diagnosis are discussed. Accepted: 11 June 1997     

Splenic hamartoma in a child in the era of PET‐CT

We present a case of a healthy 7‐year‐old female with an incidental finding of a growing splenic lesion, diagnosed as a splenic hamartoma after splenectomy. This case highlights the diagnostic challenge of splenic lesions and that the role of positron emission tomography/computerized tomography (PET/CT) in defining splenic lesions in the pediatric population remains to be defined. Pediatr Blood Cancer 2009;53:114–116. © 2009 Wiley‐Liss, Inc.     

Chondro‐osseous respiratory epithelial adenomatoid hamartoma of the nasal cavity

Chondro‐osseous respiratory epithelial adenomatoid hamartoma is a rare hamartomatous lesion of the nasal cavity, with only five cases reported in the literature to date. We report the case of a 3‐year‐old boy who presented with nasal obstruction and a mass in the left nasal cavity. The mass was completely resected on endoscopy. On microscopy, hamartomatous proliferation of respiratory‐type glands admixed with islands of immature hyaline cartilage, characteristic of chondro‐osseous respiratory epithelial adenomatoid hamartoma, was seen. Neither local recurrence nor distant metastasis was observed after 6 month follow up. Recognition of chondro‐osseous respiratory epithelial adenomatoid hamartoma as a benign lesion is important to avoid unnecessary surgical procedures.