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Resumen ANTECEDENTES: La displasia mesenquimal placentaria es una anomalía de la vasculatura y del tejido conectivo placentario que altera la estructura y la función de las vellosidades, se asocia con un desenlace perinatal adverso. CASO CLÍNICO: Paciente de 21 años, primigesta, con 15.2 semanas de embarazo establecidas por fetometría, con placenta corporal anterior, de morfología anormal, con múltiples imágenes hipoecogénicas que semejan racimos de uvas, sin flujo vascular en el Doppler color. La valoración ecográfica de las 33 semanas reportó fetometría para 27.2 semanas, anhidramnios y placentomagelia, con alteración en los parámetros de la flujometría Doppler. En la prueba sin estrés se diagnosticó restricción del crecimiento intrauterino estadio IV. La imagen del feto fue heterogénea en el flanco derecho de 4.7 x 4.1 cm. El embarazo finalizó por cesárea con una recién nacida con Apgar 8-9, de 930 gramos, placenta agrandada con vellosidades hidrópicas. El estudio histopatológico reportó: displasia mesenquimal placentaria. La resección del tumor abdominal fue electiva, por vía laparoscópica, al cuarto mes de vida, con diagnóstico histopatológico de hamartoma mesenquimal hepático. CONCLUSIÓN: La displasia mesenquimal placentaria es una anomalía placentaria benigna, relacionada con restricción del crecimiento intrauterino de inicio temprano que puede evolucionar, incluso, a la muerte del feto por hipoxia crónica debido a las anomalías en la vasculatura microscópica que afectan el desarrollo de las vellosidades terminales e interfiere en el intercambio de gases, nutrientes y electrolitos en el espacio intervelloso.
Abstract BACKGROUND: Placental mesenchymal dysplasia is an anomaly of the vasculature and placental connective tissue which alters the structure and function of the villi, being associated with an adverse perinatal outcome. CLINICAL CASE: Patient 21 years old, primigestation, with 15.2 weeks of pregnancy established by fetometry, with anterior body placenta, abnormal morphology, with multiple hypoechogenic images resembling clusters of grapes, without vascular flow in color Doppler. Ultrasound evaluation at 33 weeks, reporting fetometry for 27.2 weeks, anhydramnios and placentomagelia, with alteration in Doppler parameters, as well as in a non-stress test diagnosing fetal growth restriction stage IV. Fetus with heterogeneous image on the right flank of 4.7 x 4.1 cm. Abdominal pregnancy interruption was decided, obtaining a live female newborn, Apgar 8/9, weight 930 grams, enlarged placenta with hydropic villi. The histopathology study reports placental mesenchymal dysplasia. Abdominal tumor resection was performed electively by laparoscopy at 4 months of age with a histopathological diagnosis of hepatic mesenchymal hamartoma. CONCLUSION: Placental mesenchymal dysplasia is a benign placental anomaly, related to early-onset intrauterine growth restriction that can even progress to fetal death by chronic hypoxia due to abnormalities in the microscopic vasculature that affect the development of the terminal villi and interfere with the exchange of gases, nutrients and electrolytes in the intervillous space.
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Objective To improve the diagnostic accuracy of pediatric mesenchymal hamartoma of liver(M HL)by analyzing and summarizing the CT findings.Methods Five pediatric patients with M HL confirmed by postoperative pathology were enrolled,all patients underwent contrast-enhanced CT before operation.Results All lesions were located in the right lobe of liver.The tumor size ranged from 98 mm to 139 mm(mean size was 122 mm)in diameter.Four cases showed cystic and solid mixed masses,and one solid masses.After contrast administration,the substantial part of the mass and its septa showed enhancement while no enhancement was observed in the cystic part.No calcification was observed in the tumor.Conclusion M HL has some special CT characters.Most of M HL can be diagnosed combined with clinical practice as well as CT.
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Objective To explore the CT manifestations of pediatric mesenechymal hamartoma of liver (MHL).Methods Clinical data of 15 cases with MHL confirmed by surgery and pathology were retrospectively analyzed.All children were performed with CT scans including plain and enhanced scans,and the imaging features were analyzed.Results All the lesions were solitary,and 9 masses located at the right lobe of liver,4 located at the left lobe,the others located at both right and left lobe.The maximum diameter of lesions was from 5 cm to 30 cm,with an average of (11.52±6.84) cm.Ten cases were cystic and solid mixed,5 cases were solid.After contrast administration,the solid component and the septa of the masses showed enhancement while no enhancement was observed in the cystic component.Two cases had the spot-like calcification.Conclusion MHL has some special characteristics in the CT scan before surgery,which should be differentiated from other cystic and solid tumors of live.Some MHLs can be diagnosed when combined with the clinical information and CT images.
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Un principio básico de la patología es que las neoplasias se diferencian según sus células de origen y en el hígado semejan sus constituyentes, sean las células hepáticas, del epitelio biliar, endoteliales, mesenquimales o una combinación de estas. Es importante recordar aquí que son las metástasis el tumor maligno más frecuente del hígado, con una relación de 30:1 en pacientes sin enfermedad hepática crónica o cirrosis subyacente; es rara la presencia de las mismas en hígados cirróticos. Las neoplasias gastrointestinales del colon, páncreas, vía biliar extrahepática, estómago, tumores neuroendocrinos y GIST, o extraintestinales del pulmón, mama, melanoma o tumores de cabeza y cuello, son las más frecuentes (1). En este artículo solo revisaremos las más frecuentes. Iniciaremos con las neoplasias benignas y las lesiones pseudotumorales haciendo especial énfasis en aquellas con dificultades diagnósticas, en la utilidad de estudios especiales de inmunohistoquímica o moleculares para su adecuada clasificación y diagnóstico diferencial.
A basic principle of pathology is that neoplasms differ according to their cells of origin. Neoplasms of the liver resemble its constituent liver, biliary, epithelial, endothelial, mesenchymal cells or some combination of these different types of cells. It is important to remember here that metastases are the most frequent malignant liver tumor occurring at ratio of 30: 1 in patients without underlying chronic liver disease or cirrhosis. Metastases are rare in cirrhotic livers. The most common primary sites are the colon, pancreas, common bile duct, stomach, neuroendocrine tumors and GIST, or extraintestinal tumors from the lung, breast, head, neck and skin (1). In this article we review only the most frequent benign neoplasms and tumor-like lesions with particular emphasis on diagnostic difficulties, special studies, and the usefulness of immunohistochemical or molecular studies for proper classification and differential diagnosis.
Subject(s)
Humans , Adenoma , Angiomyolipoma , Focal Nodular Hyperplasia , Hemangioma , Neoplasms , Cysts , HamartomaABSTRACT
Rhabdomyomatous mesenchymal hamartoma is a rare skin lesion composed of a haphazard arrangement of skeletal muscle, adipocytes, and collagen. Lesions usually present as a solitary sessile papule or nodule on the head and neck in childhood. The etiology of this skin lesion is not clear, but it is thought to be caused by a migration error of the embryonic mesodermal tissues or a genetic defect predisposing to the formation of hamartoma. It may occur as part of Delleman syndrome or congenital malformations like thyroglossal duct cysts, low set ears, and sclerocornea. Herein, we present two cases of rhabdomyomatous mesenchymal hamartoma, including a 20-year-old man who had two asymptomatic erythematous papules on the chin since childhood and a 58-year-old woman with an asymptomatic solitary erythematous sclerotic plaque on the chin for 2 years. Histopathologically, the lesions exhibited normal epidermis, and mature striated muscle fibers arranged randomly within the dermis. Physical examination revealed no congenital abnormalities.
Subject(s)
Female , Humans , Middle Aged , Young Adult , Adipocytes , Chin , Collagen , Congenital Abnormalities , Dermis , Ear , Epidermis , Hamartoma , Head , Mesoderm , Muscle, Skeletal , Muscle, Striated , Neck , Physical Examination , Skin , Thyroglossal CystABSTRACT
BACKGROUND: This report describes the clinicopathologic findings of six hepatic masses that developed after Kasai hepatic portoenterostomy (HPE) in six patients with longstanding biliary atresia (BA). METHODS: Hepatic masses were found in six of 55 pediatric patients who underwent liver transplantation for BA after Kasai HPE from 1997 to 2009. Clinicopathologic analysis was performed and immunohistochemical staining was carried out for CD34, smooth muscle actin (SMA) and cytokeratin 7. RESULTS: Of the six hepatic masses, two were diagnosed as focal nodular hyperplasia (FNH)-like lesions, two were large regenerative nodules (LRN), one was a mesenchymal hamartoma (MH) and one was a cholangiocarcinoma. The immunohistochemical staining findings for SMA and CD34 were more prominent for the FNH-like nodules than for the cirrhotic background liver. Dysplastic biliary epithelium arising from intestinal metaplasia was found in the cholangiocarcinoma. CONCLUSIONS: Our findings suggest that FNH-like lesions, LRNs and MH are the results of vascular hemodynamic changes after Kasai HPE and that cholangiocarcinoma is due to recurrent cholangitis after BA. All the lesions in this series must be included in the differential diagnosis of a newly formed hepatic mass in patients after portoenterostomy.
Subject(s)
Child , Humans , Actins , Biliary Atresia , Cholangiocarcinoma , Cholangitis , Diagnosis, Differential , Epithelium , Focal Nodular Hyperplasia , Hamartoma , Hemodynamics , Keratins , Liver , Liver Transplantation , Metaplasia , Muscle, Smooth , Portoenterostomy, HepaticABSTRACT
Embryonal (undifferentiated) sarcomas arising in mesenchymal hamartoma are on record but cases of hepatic angiosarcoma (AS) arising in mesenchymal hamartoma (MH) of the liver are extremely rare. We report one such case in a 20-year-old male patient. He presented with a lump in the right hypochondriac region and pain of two years duration with rapid increase in size since two months. Ultrasonography (USG) revealed a well circumscribed mass on the undersurface of the right lobe of liver suggestive of hemangioma. The patient underwent resection of the mass. Histopathology revealed AS with areas of MH.
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El hamartoma mesenquimatoso hepático es un tumor benigno poco frecuente, formado por alteraciones de la placa hiliar y compuesto por tejidos mixomatoso, mesenquimático y conductos biliares anormales en proporciones variables. Habitualmente son formaciones hepáticas múltiples, quísticas y sin comunicación con la vía biliar. Es más frecuente en niños, es raro en adultos. El primer caso fue publicado por Yamamura et al en 1976, y se encuentran pocos en la bibliografía. Presentamos el caso de un paciente de 87 años con un hamartoma hepático quístico de 20 cm.
The mesenchymal hamartoma of the liver is a non frequent benign tumor with a ductal plate malformation, consisting of myxoid mesenchymal tissue and abnormal bile ducts in different proportion. Usually it presents as multiple liver cyst formations without communication with the principal bile duct. They are frequent in children, being rare in adults. The first case was reported by Yamamura et al in 1976, and very few cases are reported in the bibliography. We present the case of an elder 87 year old male who presented a 20 cm mesenchymal hamartoma liver cyst.
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Aged, 80 and over , Humans , Male , Hamartoma/diagnosis , Liver Diseases/diagnosis , Hamartoma/surgery , Liver Diseases/surgeryABSTRACT
Purpose: Mesenchymal hamartoma of the liver (MHL) is an uncommon benign disease of childhood, yet its biology and pathogenesis are still unknown. It is difficult to diagnose from other hepatic tumors, and malignant transformation can occur. It has caused a debate regarding choice of the proper management of each neoplasm of liver in childhood. Methods: We analyzed histologically confirmed MHLs from 1998 to 2008. Results: Six patients presented at a median age of 21months, with a painless abdominal mass. Gastrointestinal symptoms were present in two patients. Abdominal ultrasonography and CT scans revealed a cystic, septated mass within the liver in all patients. Three patients underwent major hepatic resections, one had a simple excision of the tumor, and two were observed after biopsy. The median follow up period was 46months (range 6-115months), and there were neither recurrences of tumors nor complications, in patients who underwent surgical treatment. Conclusion: Resectable mesenchymal hamartoma of the liver in childhood with normal liver function studies can be cured by complete excision without complications and without recurrence.
Subject(s)
Humans , Biology , Biopsy , Follow-Up Studies , Hamartoma , Liver , RecurrenceABSTRACT
Se reporta el caso de una paciente femenina de 21 años con diagnóstico de hamartoma mesenquimal del hígado gigante sintomático, como presentación inusual en pacientes adultos jóvenes, con tratamiento único definitivo el trasplante hepático debido al tamaño y localización anatómica del tumor. Se revisa la literatura relativa a casos reportados según edad más frecuente, métodos diagnósticos y tratamiento quirúrgico utilizados para esta patología excepcional en adultos. Palabras clave: hamartoma mesenquimal del hígado, trasplante hepático gigante.
The case of a 21 years old female patient with the diagnosis of symptomatic Gigantic Mesenchymal Hamartoma of the Liver is reported as an unusual presentation in young adult patients, with liver transplant as the only therapeutic option due to the size and anatomical location of the tumor. The literature is reviewed in relation to cases reported according to age, diagnostic methods and surgical treatment, for this exceptional pathology in adults.
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El Hamartoma mesenquimal hepático es una malformación congénita rara que en muchos casos, debido a su tamaño suele ser diagnosticado como una neoplasia verdadera. Ante esta realidad y a propósito de la presentación de un caso en nuestro hospital, nos planteamos la necesidad de definir las principales particularidades de esta patología, enfocándonos en las características imagenológicas y su correlación con la presentación clínica, patológica y epidemiológica de dicha entidad. Presentamos el caso de un niño de 18 meses de edad, que se presentó en este Instituto oncológico con un cuadro de distensión abdominal progresiva, siendo eventualmente diagnosticado de hamartoma mesenquimal hepático.
Mesenchymal hamartoma of the liver is a strange congenital malformation which in most cases, due to its size is diagnosed as a true neoplasia. In view of this reality and by means of a case report in our hospital we plant the necessity to define the principal characteristics of this pathology, focusing on imagenology and its correlation to clinical, pathologic and epidemiologic presentation of the entity. We present the case of an 18 month old boy that assisted to this hospital with progressive abdominal distention, where he was eventually diagnosed with liver mesenchymal hamartoma.
Subject(s)
Male , Infant , Congenital Abnormalities , Hamartoma , Liver , Hepatomegaly , MesodermABSTRACT
OBJECTIVE: A hepatic mesenchymal hamartoma is an uncommon benign tumor in children and little is known about the spectrum of its radiological features. The purpose of this study is to describe the spectrum of radiological features of a hepatic mesenchymal hamartoma in children. MATERIALS AND METHODS: Thirteen children with a pathologically confirmed hepatic mesenchymal hamartoma (M:F = 7:6; mean age, 3 years 2 months) were included in our study. Ultrasonography (US) was performed in nine patients including color and power Doppler US (n = 7). CT scans were performed in all patients. We evaluated the imaging findings of the hepatic mesenchymal hamartomas and the corresponding pathological features. RESULTS: Each patient had a single tumor (mean diameter: 13 cm [1.8-20 cm]). On CT and/or US, four patients (31%) had a "multiseptated cystic tumor", five patients (38%) had a " mixed solid and cystic tumor", and four patients (31%) had a "solid tumor." The septa of the cystic portion were thin in the multiseptated cystic tumors and irregularly thick in the mixed solid and cystic tumors as seen on US. On a post-contrast CT scan, solid portions or thick septa of the tumors showed heterogeneous enhancement. The amount of hepatocytes was significantly different among the three tumor groups according to the imaging spectrum (p = 0.042). CONCLUSION: A hepatic mesenchymal hamartoma in children can show a wide spectrum of radiological features, from a multiseptated cystic tumor to a mixed solid and cystic tumor, and even a solid tumor.
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Contrast Media/administration & dosage , Hamartoma/diagnosis , Liver/diagnostic imaging , Liver Neoplasms/diagnosis , Mesoderm/diagnostic imaging , Observer Variation , Radiographic Image Enhancement/methods , Retrospective Studies , Tomography, X-Ray Computed/methods , Ultrasonography, Doppler, Color/methodsABSTRACT
Cutaneous mesenchymal hamartoma is a very rare disease which most often presents itself at birth and is composed of collagen fibers, immature fibroblasts, fat cells, blood vessels, and mast cells. We report a case of cutaneous mesenchymal hamartoma on the face.
Subject(s)
Adipocytes , Blood Vessels , Collagen , Fibroblasts , Hamartoma , Mast Cells , Parturition , Rare DiseasesABSTRACT
Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood. Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4 - 32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from 10x8.5cm to 34x29cm. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion, it may be confused, and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore, complete excision of the tumor with surrounding normal liver tissue is recommended.
Subject(s)
Child , Female , Humans , Hamartoma , Liver , Recurrence , Retrospective Studies , Sarcoma , SeoulABSTRACT
Mesenchymal hamartoma is an uncommon benign tumor usually found in childhood, especially during the first two years of life. The tumor consists of a solid component and multiple cyst. Without treatment, these lesions can grow to an enormous size. We experienced a case of mesenchymal hamartoma which was found incidentally, in a 8 month old girl who had ascending cholangitis after Kasai operation due to biliary atresia. On abdominal ultrasonography and CT, there was a well-defined homogeneous small echogenic mass in the right lobe of the liver with cirrhosis. Five months later, she underwent liver transplantation. Gross picture of the resected liver showed a dark greenish pigmented solid mass in the right lobe of the cirrhotic liver. Microscopic findings showed reactive mesenchyma and epithelial overgrowth.
Subject(s)
Female , Humans , Infant , Biliary Atresia , Cholangitis , Fibrosis , Hamartoma , Liver , Liver Transplantation , Mesoderm , UltrasonographyABSTRACT
Mesenchymal hamartoma of the chest wall is a rare tumor with about 53 reported cases in the English literature. We reviewed six chest wall mesenchymal hamartomas in four patients, including two cases with multiple lesions, with specific focus on the radiologic and pathologic correlation. All cases occurred in neonates or infants with ages ranging from seven hours to seven months. They were diagnosed with plain chest radiographs (n=6), ultrasonography (n=2), chest CT scan (n=6), whole body bone scan (n=2) and MRI (n=3). All cases except a small one without cystic change showed the typical features of mesenchymal hamartoma radiographically and pathologically. Radiologically they were well-circumscribed masses with solid and cystic components with multiple fluid-fluid levels in association with single or multiple rib destruction or change. The CT scan showed the typical findings of chest wall hamartoma, and the MR showed heterogeneous signal intensities of the mass on T1- and T2-weighted images. The MR also revealed more concisely a secondary aneurysmal bone cyst formation with multiple fluid-fluid levels on the T2-weighted image. Microscopically, they showed alternating areas of cartilaginous islands and primitive appearing mesenchymal proliferation, which corresponded well with the solid component on the radiologic findings. The areas of bone formation and blood-filled cystic spaces matched the calcified or ossified densities and the cystic components, respectively. A small case without cystic change showed peculiar radiological and pathological findings resembling an osteochondroma. In conclusion, mesenchymal hamartoma of the chest wall in infancy is quite rare and sometimes can be misdiagnosed as malignancy due to the bone-destroying radiographic appearance and the highly cellular and mitotically active microscopic features, unless the radiologists and pathologists are aware of the characteristic clinical, radiological, and pathological findings. Imaging studies can usually make a correct diagnosis with good correlation to the pathologic findings.
Subject(s)
Female , Humans , Infant , Male , Hamartoma/diagnostic imaging , Hamartoma , Hamartoma/pathology , Magnetic Resonance Imaging , Mesoderm/diagnostic imaging , Mesoderm/diagnostic imaging , Mesoderm/pathology , Radiography, Thoracic , Thoracic Neoplasms/diagnostic imaging , Thoracic Neoplasms/diagnostic imaging , Thoracic Neoplasms/pathology , Tomography, X-Ray ComputedABSTRACT
Nasal chondromesenchymal hamartoma is a distinctive mixed mesenchymal lesion of sinonasal region with a complex histologic appearance and benign clinical course and clinicopathologically similar to those of the mesenchymal hamartoma of the chest wall of infancy. We report a case of nasal chondromesenchymal hamartoma occurred in the right nasal cavity in a 3-month-old female. She was admitted with a history of profuse nasal bleeding and obstruction. CT revealed complex solid and cystic mass, measuring 3.5x2.5x2.5 cm in dimensions which filled the right nasal cavity and extended into ethmoid sinus and cribriform plate. The received piecemeal fragments of tissue were brown tan-colored firm semitranslucent tissue with a cartilaginous appearance. Microscopically, the basic morphologic elements were irregular islands of hyaline cartilage and myxoid to spindle cell stroma with various cellularity.
Subject(s)
Female , Humans , Infant , Epistaxis , Ethmoid Bone , Ethmoid Sinus , Hamartoma , Hyaline Cartilage , Islands , Nasal Cavity , Thoracic WallABSTRACT
This paper presents an unusual solid mesenchymal hamartoma of the liver (MHL) in adult. A well defined solid mass in the left lobe of the liver was found in a 57-year-old female. Preoperative radiologic examinations demonstrated solid mass with multifocal calcifications abutting the gallbladder. By light microscopy, the lesion was composed of dense fibrous stroma with hyalinization, bile ducts and thick-walled vessels without hepatocytes. The solid and hyalinized mesenchymal component would suggest an unusual degenerative change representing a burnt-out MHL.
Subject(s)
Female , Humans , Hamartoma/surgery , Hamartoma/diagnostic imaging , Hamartoma/pathology , Liver Neoplasms/surgery , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/pathology , Mesoderm/pathology , Middle Aged , Tomography, X-Ray ComputedABSTRACT
An one-day-old female patient was admitted due to marked abdominal distension, cyanosis, right upper quadrant mass, and respiratory failure since birth. We applied ventilator and supportive care immediatly. However, 4 days later, she was dead due to respiratory failure and hemorragic complication. The radiographic finding showed a huge intra-abdominal mass anterior to the intestine. Autopsy findings showed a huge single cystic tumor in the right lobe of the liver. The cysts were filled with sanguinous fluid. There wasn't any signs of necrosis or calcification. Microscopically, the tumor was well demarcated from the adjacent hepatic parenchyma. It consists of connective tissue with dilated lymphatics, vessels, and bile ducts.
Subject(s)
Female , Humans , Autopsy , Bile Ducts , Connective Tissue , Cyanosis , Hamartoma , Intestines , Liver , Necrosis , Parturition , Respiratory Insufficiency , Ventilators, MechanicalABSTRACT
Cutaneous mesenchymal hamartoma (CMH) is a very rare disease which most often presents itself at birth and is composed of collagen fibers, immature fibroblasts, fat cells, blood vessels, and mast cells. Until recently, only two cases have been reported. Thus, the clinical and histopathological findings have been unsettled. We describe three cases of cutaneous mesenchymal hamartoma with different clinical findings, discuss its pathogenesis, and suggest that CMH is a distinct disease entity.