Mesenchymal hamartoma of the chest wall may be recognized by its characteristic occurrence in infancy and crosssectional imaging features of mineralization and hemorrhagic cystic secondary abc. Imaging plays crucial roles in the management of pediatric patients with suspected liver malignant tumors. Pediatric mesenchymal hamartomas of the liver can show both. For 14 mesenchymal hamartomas of the chest wall in 12 children, radiologic studies computed tomographic ct scans n 14, radiographs n 11, magnetic resonance mr images n 9, and bone. Well circumscribed, solitary myxoid mass with fluid filled cysts, with architecturally abnormal bile ducts in primitive myxoid stroma arch pathol lab med 2006. Previously considered a developmental anomaly, newer insights into other theories of origin including toxicmetabolic, ischemic, and a true neoplastic process are in progress. Fineneedle aspiration cytology of mesenchymal hamartoma of the chest wall. Mesenchymal hamartoma of the liver pubmed central pmc. Our patients database over the past 25 years and all articles available from pubmed up to december 2016 were. Each case has a different clinical course as well as different radiological imaging findings, and various histopathological diagnoses. The mass showed lack of enhancement on mri and was photopenic on petct. Mesenchymal hamartoma mh is not an uncommon tumor of the liver in the age group of 210 years. The hamartoma is the dark circular object on the left that dominates the image. Mesenchymal hamartoma of the liver mhl is an uncommon tumour composed of architecturally abnormal bile ducts in an uncommitted myxoid stroma.
Herein we report a mesenchymal hamartoma with rhabdomyomatous features occurring in the orbit. As a result, we report the imaging findings for two cases of pancreatic hamartoma. Anupindi 1, peter mattei 2, anita sengupta 3, kassa darge 1 1. Mesenchymal hamartoma also occurs in children, and its imaging appearance depends on the presence of stromal elements and the protein. You may need to register to view the medical textbook, but registration is free.
Mesenchymal hamartoma mri of the liver doctor steve abel. The hamartoma is characterized by marked overgrowth of mesenchymal or connective tissue of varying maturity and marked tendency to cyst formation. This report describes a case of mesenchymal cystic hamartoma of the liver with diagnostic imaging workup using only ultrasound and mri. To describe the imaging features of chest wall mesenchymal hamartoma with emphasis on crosssectional imaging and comparison with histopathologic results. This report presents a neonatal case of mesenchymal hamartoma of the bilateral chest wall. Hk j paediatr new series hong kong journal of paediatrics. Preoperative radiological examination revealed a large. In fact, some authors believe that a mesenchymal hamartoma represents a benign counterpart of an embryonal cell carcinoma. Mesenchymal hamartomata in the chest wall are extremely infrequent benign tumors that tend to appear during early infancy, and their presence in the prenatal period is exceptionally rare 4. Clinical findings haemoptysis, pneumothorax, haemothorax, pleuritic chest pain, dyspnoea, or combinations.
Summary mesenchymal hamartoma of the liver mhl is a benign tumor affecting children that is characterized by a primitive myxoid stroma. A mesenchymal hamartoma of the chest wall is a rare benign tumor. Shows some overlapping features with pulmonary chondroma of carney triad, but hamartomas tend to be single rather than multiple, favor men rather than women, and show entrapped respiratory epithelium and fat, without a fibrous pseudocapsule. Mesenchymal cystic hamartoma definition of mesenchymal. Cystic epithelium in mesenchymal hamartoma mh is typically biliary. Pulmonary chondroma sometimes used interchangeably with pulmonary chondromatous hamartoma, but true. Hepatoblastoma with solid and multicystic aspect mimicking a mesenchymal hamartoma. Pubmed is a searchable database of medical literature and lists journal articles that discuss rhabdomyomatous mesenchymal hamartoma. Mesenchymal hamartoma of the chest wall in infants and children. Ultrasonographic diagnosis of a fetal abdominal mass. The mesenchyme may contain minor remnants of liver cells, duct cells and even portal tissue, the most characteristic feature being overgrowth of connective tissue. Mesenchymal hamartoma of liver is a common benign pediatric tumor usually seen in male infants by the age of 1 year. A hepatic mesenchymal hamartoma is an uncommon benign tumor in children and little is known about the spectrum of its radiological features.
Mesenchymal hamartoma radiology discussion including radiology cases. A single septal calcification associated with this tumor was demonstrated, an association which has not previously been reported. Once found and confirmed as a hamartoma based on typical findings on mammography andor ultrasound, the finding does not usually require followup imaging or surgery. One 57yearold female patient and one 69yearold male patient presented with pancreatic lesions incidentally detected by us. Mesenchymal hamartoma of the liver identified by fetal sonography. Hepatic mesenchymal hamartomas typically occur in children and neonates 3 with. Epithelial and mesenchymal components branching bile ducts without atypia in loose, myxoid stroma with myofibroblastlike cells, dilated vessels and lymphatics may resemble breast fibroadenoma at low power. Ulnar nerve lesions typically have a crosssectional area of 0. Herein we report a mesenchymal hamartoma with rhabdomyomatous features occurring in.
The angle of 3 d images composed by most software programs can be. Ct and mr imaging of benign hepatic and biliary tumors. Final pathology revealed that the tumor was a mesenchymal hamartoma of the liver. However, since malignancy of the lesion could not be ruled out. Mesenchymal hamartomas, which typically occur in children under 2 years of age, present as a large solitary neoplasm with variable amounts of solid and cystic components on ultrasound or computed tomography 8, 9. Rhabdomyomatous mesenchymal hamartoma genetic and rare.
Clinicians guide to imaging and pathologic findings in benign breast disease. Hepatic mesenchymal hamartoma are rare benign tumors. Nicholson sa, hill da, foster kw, mcalister wh, davila rm, dehner lp. Mesenchymal hamartoma of the liver is a rare benign tumor of childhood which has been confused with various other benign liver lesions, particularly vascular hamartomas which are mesodermal but not mesenchymal. Typically, it presents as a large benign multicystic liver mass in children younger than three years, amenable to complete resection. Mr imaging with pathologic correlation michael moore 1, sudha a. Grossly, mhl is a solitary mass with cystic spaces of variable size. Ruhland department of obstetrics and gynecology, division of prenatal medicine, university hospital of schleswig.
They infrequently develop in late infancy, and the oldest age at diagnosis reported is. So if your radiologist says hamartoma, take a relaxing breath its just a benign growth of normal breast tissue. In fibrous hamartoma of infancy, bundles of welldefined thick connective tissue, which branch, interweave, and project into adjacent. Hepatoblastoma with solid and multicystic aspect mimicking. Mesenchymal hamartoma of the liver and dicer1 syndrome. Radiological spectrum of hepatic mesenchymal hamartoma in. Medscape reference provides information on this topic. Mesenchymal hamartoma pediatric radiology reference. Cross sectional imaging of mesenchymal cystic hamartomas is often necessary to help facilitate planning of surgical resection. Groom kr, murphey md, howard lm, lonergan gj, rosadodechristenson ml, torop ah. The purpose of this study is to describe the spectrum of radiological features of a hepatic mesenchymal hamartoma in children. Paediatric undifferentiated embryonal sarcoma ues and mesenchymal hamartoma mh of liver have similar imaging cystic imaging characteristic but have totally different clinical behaviour.
Mesenchymal hamartoma of the liver is the second most common benign liver tumor in children, yet its biology and pathogenesis are poorly understood. The tumors are generally welldefined and encapsulated or pedunculated. Holstein, campus luebeck, ratzeburger allee 160, 23538 luebeck, germany. We present the findings on fetal us and mri of a histologically confirmed case. This report describes a case of a well defined solid mass in the right lobe of the liver in a 51 year old man. The hamartomas were assessed for interlobular fibrotic stroma, stromal adipose tissue content, pseudoangiomatous stromal hyperplasia, and epithelial changes hyperplasia, adenosis. Mesenchymal hamartoma of bone portnotes orthopaedicsone. We present a multiseptated mesenchymal hamartoma of the liver in a 10yearold male patient, a rare benign tumor of childhood. Noncontrast ct scan shows a lowattenuation mass in the right. Citeseerx document details isaac councill, lee giles, pradeep teregowda. The usual presentation is with a nontender abdominal mass. Angiography dsa while not being a standard diagnostic imaging modality of choice, angiography may show peripheral hypervascularity to the lesion with a septated avascular center 39. Prenatal and postnatal diagnosis by imaging alicia martinezvarea, 1 jose maria vilavives, 1 juan jose hidalgomora, 1 antonio abadcarrascosa, 1 roberto llorenssalvador, 2 and alfredo peralesmarin 1. Lipomas and fibroadenolipomas hamartomas are benign, mesenchymal lesions, and they are usually readily diagnosed with conventional imaging.
Radiological spectrum of hepatic mesenchymal hamartoma in children objective. Mesenchymal hamartoma of the liver occurs almost exclusively in infancy and childhood, with approximately 140 total cases reported. Mesenchymal hamartoma of the liver case reportand short. Mesenchymal hamartoma of the liver mhl is an uncommon cystic mass of infancy that is a developmental anomaly rather than a neoplasm.
Fibrous hamartoma of infancy is a benign mesenchymal tumour of myofibroblastic derivation with prototypical histopathologic features. Radiologic manifestations with emphasis on crosssectional imaging and histopathologic comparison. Orbital mesenchymal hamartoma with rhabdomyomatous features. It will remain a unique feature of a persons breast. Nov 15, 2019 on imaging studies mesenchymal hamartoma is a large, predominantly cystic mass frequently measuring 15 cm or more in diameter at the time of diagnosis. The clinical picture includes gastrointestinal symptoms, weight loss, and anorexia. Pulmonary hamartoma is a benign pulmonary neoplasm composed of mesenchymal tissues including cartilage, fat, connective tissue and smooth muscle. Hepatic mesenchymal hamartoma radiology reference article.
Mesenchymal hamartoma is the second most frequent benign liver tumor in young children and constitutes approximately 8% of all tumors in this population, with 80% of these tumors presenting within the first two years of life. Mesenchymal hamartoma of the liver mhl is a rare primary hepatic tumor composed of a complex mixture of immature mesenchyme interspersed with small abnormal bile ducts, aberrant blood vessels. In addition, mesenchymal hamartoma is sometimes mistaken for a malignant tumor. Hepatic mesenchymal hamartoma hmh is the second most. Pulmonary hamartoma ph is the most common benign tumor of the lung and is the third most common cause of solitary pulmonary nodules. The predominantly cystic appearance of the tumor was consistent with a cystic liver tumor, namely mesenchymal hamartoma. Fineneedle aspiration cytology of mesenchymal hamartoma of the. Most mhl are diagnosed in childhood and few cases have been reported in adults. With the development of medical imaging, 3d reconstruction technology, the innovation of liver surgery and the proposal of precise hepatectomy, the. Adult mesenchymal hamartoma of the liver mimicking bile duct. The mesenchyme may contain minor remnants of liver cells, duct cells and even portal tissue, the most characteristic feature being overgrowth of. Serum afp levels may be normal or markedly elevated. Mesenchymal hamartoma of the liver and dicer1 syndrome nejm. Mr imaging appearance of mesenchymal hamartoma can also vary depending on the presence of stromal elements as well as the protein content of the fluid 8.
Large multilobulated complex cystic mass in the right aspect of the abdomen, extending across midline to the left side along mid abdomen. Threedimensional 3d imaging could significantly improve the resection rate of pediatric tumors and increase the safety of the surgery. Symptoms of hamartoma depend on the location of the tumor. Tumours usually present as solitary masses on the limbs, trunk, sacrum, or scrotum. If located endobronchially, they can cause irritation of bronchial mucosa resulting in persistent cough as a presenting symptom. We report a rare case of mesenchymal hamartoma in the cirrhotic liver of a 52yearold japanese male. It makes up approximately 8% of all pediatric liver tumors. There are numerous internal septations, which are better appreciated on prior ultrasound. Accurate diagnosis of mesenchymal hamartoma is important since many chest wall. Patients are seen initially with painless progressive abdominal enlargement. Pawel br, crombleholme tm 2006 mesenchymal hamartoma of the chest wall.
It is the second most common benign liver tumor in children. The imaging appearance in young children is similar to that seen with an undifferentiated embryonal cell carcinoma. Rhabdomyomatous mesenchymal hamartoma rmh is a subcutaneous lesion that is characterised by a normal epidermis overlying a dermis that contains randomly arranged mature striated muscle fibers associated with varying amounts of mesenchymal elements such as adipose tissue, collagen and blood vessels. The clinical presentation of thoracic mesenchymal hamartomas varies from an asymptomatic chest wall mass to severe respiratory distress resulting from compression of the airways and lungs. Hepatoblastoma with solid and multicystic aspect mimicking a. We conducted a comprehensive search of the database of our vascular anomalies center over the past 12 years 19992010 for the keywords mesenchymal hamartoma, liver, and hemangioma in a large cohort of patients with liver hemangioma. Dec 17, 2018 pulmonary hamartoma ph is the most common benign tumor of the lung and is the third most common cause of solitary pulmonary nodules. Following surgical resection, pathological examination corresponded to the crosssectional imaging features with haemorrhagic. To describe the imaging features of hepatic mesenchymal hamartoma with emphasis on magnetic resonance imaging mri compared to histopathologic results. What is the appearance of lipoma and hamartoma on magnetic. Entrapped respiratory epithelium may also be found within the lesion. Ros pr, goodman zd, ishak kg, dachman ah, olmsted ww, hartman ds, lichtenstein je. Mr imaging with pathologic correlation moore et al. The initial prospective pathological examination of the mass did not identify any calcification.
Orbital mesenchymal hamartoma with rhabdomyomatous. Patients can be asymptomatic with peripheral parenchymal hamartomas, with incidental discovery on chest imaging obtained for another reason. Bile duct cystadenoma was considered most likely preoperatively because of the patients age and the normal levels of tumor markers. Because cystic change, calcification, and contrast enhancement at mr imaging can be observed in hamartoma, prasad et al 4 reported that cystic changes in hamartoma are caused by hemorrhage or fig 1. Spinecho sequencese,fast spinecho sequencefse were detected in 12 children 7 males,5 females with mesenchymal hamartoma of the. Mesenchymal hamartoma of the chest wall may be recognized by its characteristic occurrence in infancy and crosssectional imaging is needed to differentiate it from fibrous hamartoma 3.
Mesenchymal hamartoma of the chest wall in infants. Previous understanding of a purely benign nature of the. Mesenchymal hamartoma of the bilateral chest wall in. Mesenchymal cystic hamartoma of the liver is a rare benign developmental malformation comprised of multiple cysts with internal septae. Jimenezheffernan ja, vicandi b, lopezferrer p, et al. We demonstrate an approach starting from the pediatricians. Webpathology is a free educational resource with 10363 high quality pathology images of benign and malignant neoplasms and related entities. Videoassisted thoracoscopic surgery and resection of the mass was performed and showed a mesenchymal cystic hamartoma invading the left hemidiaphragm.
Imaging evaluation of liver tumors in pediatric patients. On imaging studies mesenchymal hamartoma is a large, predominantly cystic mass frequently measuring 15 cm or more in diameter at the time of diagnosis. Total excision of congenital mesenchymal hamartoma of the. Mesenchymal hamartoma of the liver fetal ultrasound. Radiological spectrum of hepatic mesenchymal hamartoma. On crosssectional imaging, lipofibromatous hamartoma of the ulnar nerve can be more difficult to appreciate than is lipofibromatous hamartoma involving the median nerve.
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