Mesenchymal Cystic Hamartoma Research Articles

USCAP Specialty Conference, Case 1: Type I Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) was defined in 1988 by Manivel et al. in a series describing 11 intrathoracic pulmonary neoplasms in young children. The PPB is a unique peripheral pulmonary or pleural-based tumor of childhood that is characterized in its earliest form as a bland-appearing multiloculated cyst with small foci of tumor cells and in later forms as mixed and predominantly primitive, overtly malignant neoplasms. Prior to the introduction of the PPB as a distinct entity, this tumor had been reported in the literature as pulmonary blastoma, sarcoma arising in mesenchymal cystic hamartoma, embryonal sarcoma, malignant mesenchymoma, primary pulmonary rhabdomyosarcoma and rhabdomyosarcoma arising in congenital adenomatoid malformation or bronchogenic cyst. Over the past 15 years, PPB has come to be recognized in centers around the world. With the establishment of the Pleuropulmonary Blastoma Registry by Jack Priest, MD, and colleagues, there has been improved understanding of this rare pediatric neoplasm.

Mesenchymal cystic hamartoma of the lung in Cowden's disease

Mesenchymal cystic hamartoma of the lung is a rare clinicopathological entity (1), characterized by a cystic pulmonary lesionwith typical pathological features, that slowly increases in size. Fewer than 15 cases have been reported in the literature. Cowden’s disease, or the multiple hamartoma syndrome, is an autosomal dominant tumor syndrome with involvement of various organs. Clinically, it is characterized by multiple hamartomas of ectodermal, endodermal, and mesodermal origin, a high incidence of malignant tumors of the breast, thyroid gland, and female adnexa together with gastrointestinal polyps, various mucocutaneous lesions, macrocephaly, and mild-to-moderate mental retardation (2). Our present observation suggests that Cowden’s disease may include mesenchymal cystic hamartoma of the lung.

Fetal therapy for giant hepatic cysts

Cystic mesenchymal hamartoma is an extremely rare, benign tumor. Rapid growth to a giant size can pose a threat not only in early childhood but also during fetal life. The experience with 2 antenatally diagnosed giant hepatic cysts with widely disparate approaches to management, treatment, and outcome is presented. A giant hepatic cyst was diagnosed on routine screening ultrasound scan. Because of its extremely massive size, the cyst was treated in utero with repeated aspirations, primarily for obstetric considerations. The infant did well, and the lesion was excised laparoscopically during the neonatal period. A second fetus with a giant hepatic cyst was not treated in utero, and the pregnancy continued to term. Nonimmune hydrops fetalis developed, and the fetus was delivered prematurely at 34 weeks. At birth, the infant was noted to have diffuse neurologic injury and no urine output despite normal-appearing kidneys. The lesion was excised during the neonatal period by open laparotomy. Observations at the time of surgery and pathologic studies of the placenta showed aneurysmal dilatation of the placental veins suggesting in utero compression of the fetal intraabdominal umbilical vein. The infant died shortly after birth. The experience with these 2 cases suggests the possibility that giant mesenchymal hamartoma diagnosed in utero may cause umbilical venous obstruction leading to ischemia during fetal life. Decompression of giant hepatic cysts may reverse this phenomenon and allow normal fetal development. J Pediatr Surg 37:E31. Copyright 2002, Elsevier Science (USA). All rights reserved.

Cystic Mesenchymal Hamartoma Mimicking Hepatic Hydatid Disease

Hydatid disease, caused by Echinococcus granulosus, is relatively rare in children. It is often described as having a pathognomonic appearance on ultrasound (US) and computed tomography (CT) [1]. Mesenchymal hamartoma is a rare hepatic developmental abnormality con®ned almost exclusively to children under the age of 2 years. After haemangioma it is considered to be the second commonest benign liver tumour in the paediatric age group, with 140 cases reported up to 1990 [2]. We report a case of cystic mesenchymal hamartoma in a 7-year-old girl which was radiologically indistinguishable from hepatic hydatid disease.

Mesenchymal Hamartoma of the Liver

=Abstract=Mesenchymal hamartoma of the liver is an uncommon benign lesion that appears as a large abdominal mass, almost exclusively in the first 2 years of life. It has been believed that this lesion is not a true neoplasm but rather a kind of hamartomatous lesion arising from the connective tissue of the portal tracts at some point in their development. Minor components of liver cells and bile ducts are considered a result of entrapment. We report a case of typical cystic mesenchymal hamartoma in a 17-month-old boy who first presented with an abdominal mass at 7 months of age. The tumor was located in the right lobe, 17 cm in maximum diameter, and was largely cystic and partly solid, The cysts were multiloculated and lined by a single layer of tall columnar partly ciliated epithelium, and the irregular admixture of mature liver cell islands and intervening loose myxoid stroma was evident. Electronmicroscopic study revealed that the lining epithelium was the same as that of the bile duct, and the liver cell element was so mature that it couldn't be differentiated from normal hepatocytes. The stroma cell resembled fibroblast.

Endometrial stromal tumors: an update on a group of tumors with a protean phenotype.

Endometrial stromal tumors are reviewed with emphasis on their wide morphologic spectrum and problems in differential diagnosis, highlighting issues that have received particular attention in the recent literature. These neoplasms are divided into two major categories--endometrial stromal nodules and endometrial stromal sarcomas--a distinction made on the basis of the lack of significant infiltration at the periphery of the former. The division of endometrial stromal sarcomas into low-grade and high-grade categories has fallen out of favor and the designation endometrial stromal sarcoma is now considered best restricted to neoplasms that were formally referred to as "low-grade" stromal sarcoma. Endometrial sarcomas without recognizable evidence of a definite endometrial stromal phenotype, designated poorly differentiated "endometrial sarcomas," are almost invariably high grade and often resemble the mesenchymal component of a malignant mullerian mixed tumor. Two features of endometrial stromal tumors that may cause confusion are smooth muscle differentiation and epithelial patterns. Cases in the former category often have a characteristic "starburst" pattern of collagen formation. The most common epithelial patterns resemble those seen in ovarian sex-cord stromal tumors. Much less common is endometrioid gland differentiation. Some endometrial stromal tumors have a prominent fibrous or myxoid appearance and the myxoid tumors should be distinguished from myxoid leiomyosarcoma. Other unusual features of endometrial stromal tumors are also discussed. Lesions in the differential diagnosis of uterine endometrial stromal neoplasms include highly cellular leiomyoma, cellular intravenous leiomyomatosis, adenomyosis with sparse glands, metastatic carcinoma, and lymphoma. Endometrial stromal sarcomas at extrauterine sites may be primary or metastatic from a uterine tumor, the latter sometimes being occult and difficult to definitively establish, particularly if there is a history of a remote hysterectomy for "leiomyomas." Endometrial stromal sarcomas of the ovary, whether primary or metastatic, may be difficult to distinguish from ovarian sex-cord stromal tumors. Extragenital endometrial stromal sarcomas may be confused with diverse lesions such as gastrointestinal stromal tumors, hemangiopericytoma, lymphangiomyomatosis, or mesenchymal cystic hamartoma of the lung. Immunohistochemistry may play a role in evaluating these tumors and in some instances establishing the diagnosis although conventional light microscopic analysis suffices in the majority of cases. The unusual tumor, the "uterine tumor resembling an ovarian sex-cord tumor," is also considered in this review as it is almost certainly of endometrial stromal derivation in many cases. These neoplasms may have a striking resemblance to granulosa cell tumors or Sertoli cell tumors, including those with a retiform pattern, and have recently been shown to be frequently inhibin positive.

Mesenchymal Hamartoma of the Liver in Beckwith-Wiedemann Syndrome: The First Reported Case

The Beckwith-Wiedemann syndrome is a rare condition characterized by macrosomia, macroglossia, omphalocele, and visceromegaly. Although the development of neoplasia is frequent, the English literature has never described an association with mesenchymal hamartoma of the liver. We report an exceptional case of Beckwith-Wiedemann syndrome associated with a cystic mesenchymal hamartoma of the liver in a newborn.

Fatal haemorrhage from mesenchymal cystic hamartoma of the lung

A 34 year old women was admitted to the hospital with a 9 year history of intermittent haemoptysis associated with increasing breathlessness. A working diagnosis of lymphangioleiomyomatosis was made, based on clinical, radiological and histological findings. Three years later, the patient was admitted to hospital with worsening haemoptysis, which rapidly progressed and resulted in death from massive pulmonary haemorrhage. Postmortem examination and histology revealed findings consistent with multiple mesenchymal cystic hamartomas of the lungs. This is a rare condition which has previously been described as having a good prognosis. This case is the first fatality resulting directly from the disease to be reported.

The ultrasonographic appearance of a cystic mesenchymal hamartoma of the liver observed in a middle‐aged woman

The ultrasonographic appearance of a cystic mesenchymal hamartoma of the liver observed in a middle‐aged woman

Mesenchymal Cystic Hamartoma of the Lung

We report the case of an asymptomatic 51-year-old man, presenting with chest X-ray abnormalities consisting in multiple pulmonary nodules; the diagnosis of mesenchymal cystic hamartoma of the lung was made by an open lung biopsy, revealing both nodules of mesenchymal cells and cysts with a lining of normal or metaplastic epithelium. Clinical and pathological characteristics of this rare entity are reviewed.

Pediatric case of the day. Pulmonary mesenchymal cystic hamartoma.

Pediatric case of the day. Pulmonary mesenchymal cystic hamartoma.

A giant cystic mesenchymal hamartoma of the liver: Diagnosis, management, and study of cyst fluid

A 21-yr-old West Indian woman with a giant hepatic cyst is reported. Preoperative investigations revealed that it was not a simple congenital cyst but that it was highly vascular. Studies of the cyst fluid obtained by percutaneous catheter drainage suggested that the epithelial lining was of bile duct origin. Fluid output was increased by secretin administration, but remained unchanged after amiloride. Bromsulphthalein did not enter the cyst fluid after intravenous injection. A hemihepatectomy was performed and the histologic appearances of the lesion were consistent with cystic mesenchymal hamartoma, a rare lesion in this age group. The patient is well 4.5 yr later.

Cystic Hamartoma of the Liver in a Pediatric Patient

A case of cystic mesenchymal hamartoma in a 2-year-old boy is presented. The overall angiographic appearance closely resembled that of the more common renal angiomyolipoma, with the addition of multiple focal avascular areas representing the cystic component of the lesion. The coexistence of these angiographic findings in a young patient with an otherwise asymptomatic upper abdominal mass should suggest cystic mesenchymal hamartoma.

Congenital cysts of the liver in infancy and childhood

A review of congenital cysts of the liver is made, including cases from the Childrens Hospital of Los Angeles, and those cases of cystic mesenchymal hamartoma and non-parasitic cysts in infancy and childhood which have been reported in the medical literature. The gross surgical findings and the operative procedures are emphasized, so that the surgeon encountering these lesions will be better able to decide on the nature of the lesion and on a safe and indicated operative procedure.