A distinctive myoepithelial hamartoma of the pancreas histologically confirmed in the mother of a previously reported patient

We encountered a 62-year-old female patient with a distinctive pancreatic myoepithelial hamartoma characterized by dilated loops formed by pancreatic branch ducts. The patient, who experienced recurrent acute pancreatitis caused by pancreatic juice stasis, underwent subtotal stomach-preserving pancreatoduodenectomy, achieving remission of pancreatitis. Computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) demonstrated a honeycomb appearance of the pancreatic head, consisting largely of loop-forming dilated pancreatic branch ducts. Radiography of resected specimens demonstrated a tortuous main pancreatic duct that narrowed in the head, but communicated with the pancreatic branch ducts forming intricate loops within the lesion. Histologic examination showed dilated pancreatic ducts embedded in thick layers of smooth muscle, leading to a diagnosis of myoepithelial hamartoma. Her son shared a similar clinical course, radiologic findings, and histopathologic findings with his mother. MRCP demonstrated a honeycomb appearance of the pancreatic head in her daughter, who complained of persistent diarrhea. To our knowledge, this is the first English-language reports of such a myoepithelial hamartoma of the pancreas showing familial occurrence.     

Mesenchymal cystic hamartoma of the lung: A case report

Rationale:Mesenchymal cystic pulmonary hamartoma is a rare type of hamartoma that has been reported in all cases in the literature. Most patients were reported to have spontaneous pneumothorax and were treated by surgery, and finally confirmed to be caused by rupture of the cystic hamartoma. Here, we report a case of mesenchymal cystic pulmonary hamartoma detected using computed tomography (CT) during a health check-up without obvious symptoms.Patient concerns:A 60-year-old woman was detected using CT during her health check-up. She was a non-smoker and had no symptoms or history of specific diseases.Diagnosis:The final pathological examination confirmed that the lesion was a mesenchymal cystic hamartoma of the lung.Interventions:A uniportal video-assisted thoracic surgery wedge resection was performed for biopsy.Outcomes:The patient recovered smoothly and was discharged on postoperative day 3.Lessons:For cystic pulmonary hamartoma, it is usually difficult to make a correct diagnosis using CT imaging. A chest magnetic resonance imaging examination may be helpful for differentiation diagnosis before video-assisted thoracic surgery biopsy.     

Tumors of the small intestine with little or no malignant predisposition: a review of the literature and report of 56 cases

Benign small bowel tumors are rare. If those with malignant potential are excluded, a small group of truly benign lesions remains. Fifty-six cases of these small bowel tumors were analyzed for clinical data, pathologic diagnosis, number, size, location, and radiologic appearance. Lesions included lipoma, myoepithelial hamartoma, Peutz-Jeghers hamartoma, neurogenic tumors (including gangliocytic paraganglioma), Brunner's gland abnormalities, and inflammatory fibroid polyp. Location, number, and radiographic morphology can be helpful in reaching a more specific diagnosis. Age of patient and size of tumor may be helpful in the differential diagnosis; however, sex of the patient and clinical symptoms are not.     

Laser-assisted resection of a giant pulmonary chondrohamartoma--a case report

A rare case of a giant pulmonary chondromatous hamartoma (15 cm, 1350 g) resected by a new laser system (Nd:YAG, 1318 nm, 40 W) is presented. The laser management of a hamartoma resection--the largest reported to date in the literature--is presented here.     

Mesenchymal hamartoma of the liver in an adult: radiologic diagnosis

A case of mesenchymal hamartoma of the liver in a 30-year-old female is reported. Ultrasonography, computed tomography, and hepatic angiography demonstrated the presence of a large, mixed cystic and solid mass within the liver, suggesting the diagnosis of a mesenchymal hamartoma. At surgery, the tumor proved to be unresectable.     

Mature cardiac myocyte hamartoma in the right atrium

During coronary artery bypass grafting in a 58-year-old man, a mass was discovered incidentally in the right atrium, measuring 1.5 x 1 x 0.5 cm. It was composed of disorganized hypertrophic mature cardiac myocytes, and associated with focal fibrosis, mature adipocytes, and mild lymphocytic infiltration in peripheral areas, indicative of cardiac hamartoma. This type of hamartoma has been rarely reported as an isolated mass in the right atrium.     

Clinical Presentation and Anatopathologic Finding of a Hepatic Vascular Hamartoma: a Case Report.

HVH (hepatic vascular hamartoma) is a tumor like malformation arising from the vascular tissue of the liver. HVH has been previously reported in animals and presents distintive features from the most frequent benign tumor like malformation of the liver, the hepatic mesenchymal hamartoma (HMH). Herein we report a case of HVH localized in hepatic segment 4b, involving the gastro hepatic ligament, successfully treated with total excision. We describe the anatomo-pathologic findings focusing on the clinical and radiological presentation, the intraoperative characteristics and the differential diagnosis.     

A spinal hamartoma in an aged woman

Spinal harmatoma is an extremely rare tumor, and only 16 cases have been reported previously. We report on a 73-year-old woman with low back pain because of spinal hamartoma. To our knowledge, the current report is the first published case of a spinal hamartoma without other systemic diseases in the aged.     

Giant mesenchymal hamartoma of the liver in an adult

We report a case of hepatic mesenchymal hamartoma in an adult; this condition is extremely rare, with only 15 cases having been reported in the English-language literature worldwide. The patient was a 36-year-old woman who was seen at her local hospital for upper abdominal distension. A giant multilocular cystic tumor, which had almost entirely replaced the normal parenchyma of the right lobe of the liver, was diagnosed. She was referred to our hospital, where, with a diagnosis of biliary cystadenoma, the tumor was successfully removed by right hemihepatectomy. After an uneventful postoperative course, the patient was discharged from our hospital. On histological examination, the tumor consisted of numerous cystic lesions without epithelial lining cells; hepatocytes, bile duct, and vascular components, without either lobular structure or atypia, were observed in the pseudocyst wall, leading to a diagnosis of hepatic mesenchymal hamartoma. There have been a few previously reported cases of multifocal hepatic mesenchymal hamartoma reappearing in the remaining liver after hepatectomy, although these cases are considered to be extremely rare. Therefore, periodic follow-up will be necessary for the patient.     

Pulmonary hamartoma syndrome

We reviewed 24 charts of patients with pulmonary hamartoma; numerous congenital anomalies and benign tumors were observed in this series. Most of the observed associations are known to occur in patients with Cowden's syndrome, which is characterized by multiple hamartomatous neoplasms of ectodermal, mesodermal, and endodermal origin; however, to our knowledge, there has been no reported association between pulmonary hamartoma and Cowden's syndrome. We conclude that pulmonary hamartomas are frequently accompanied by other developmental abnormalities and benign tumors.     

Endobronchial hamartoma

Two cases of endobronchial hamartoma are reported, making a total of forty-two noted in the British and American literature. Although uncommon, hamartoma should be given consideration in any case manifesting bronchial obstruction.     

Adenomyomatous Hamartoma of the Small Intestine: A Rare Cause of Intussusception in an Adult

A case of small intestinal obstruction in an 82-yr-old woman caused by an ileoileal intussusception is reported. At the leading point of the intussusceptum an adenomyomatous hamartoma was found. In a review of the literature we were unable to find a similar case.     

气管-支气管肺肌上皮瘤临床分析

目的探讨气管-支气管肺肌上皮瘤临床表现、诊断及治疗。方法回顾性分析本院1例、近15年国内外报道的10例确诊的气管-支气管肺肌上皮瘤病例资料、随访记录。结果发现气管-支气管肺肌上皮瘤多表现咳嗽、气促、咯血;确诊及治疗依靠手术。结论气管-支气管肺肌上皮瘤是一种罕见的肺部肿瘤,手术切除足治愈本病的主要手段。     

Bronchoscopic features and bronchoscopic intervention for endobronchial hamartoma

Background and objective: Bronchoscopic resection of endobronchial hamartomas has been reported to have a favourable outcome. This study describes the bronchoscopic features of endobronchial hamartoma and reports the clinical outcome of bronchoscopic intervention. Methods: A retrospective analysis was conducted of patients with histologically proven endobronchial hamartomas, diagnosed in the 10‐year period 1999–2009 to elucidate the clinical, radiological and bronchoscopic features of hamartoma and to describe the clinical outcomes. Results: Seventeen of the 135 patients with pulmonary hamartomas were diagnosed as having endobronchial hamartomas. CXR was abnormal in 11 of the 17 patients. On chest CT (n = 16), the median diameter of the lesion was 15.6 mm. Calcification and areas of focal fat in the lesion, the diagnostic CT findings of pulmonary hamartoma, were found in two of 16 (12.5%) patients. At bronchoscopy (n = 16), all tumours had a mass appearance and most were smooth surfaced round masses (50.0%) with 18.8% having a ‘stalk’. Bronchoscopic forceps biopsies were performed in 13 patients, which resulted in five patients (38.5%) being diagnosed with endobronchial hamartoma. Fifteen patients were treated with rigid or flexible bronchoscopic resection, one had lobectomy, and one had no intervention. No procedure‐related mortalities or late complications developed. Conclusions: Bronchoscopic intervention appears to be a safe and effective method to resect endobronchial hamartomas.     

Mesenchymal hamartoma of the liver.

The case of a 19 yr-old female with abdominal pain, jaundice, and hepatomegaly is reported. A large mass replacing the left hepatic lobe, detected by scintigraphic and angiographic studies and also by means of ultrasound and transhepatic radio-opaque filling, proved to be cystic. A left hepatic lobectomy was performed. Pathologic examination revealed a mesenchymal hamartoma, a rather rare event in view of the patient's age. Prompt recovery followed surgery, and the patient is well and symptomless 1 yr after operation.     

Splenic hamartoma associated with thrombocytopenia

A case of splenic hamartoma associated with thrombocytopenia is reported. A 70-year-old man was referred to our hospital because of carcinoma of the body of the pancreas. Hematological examination disclosed thrombocytopenia and elevated serum CA19-9 and Span-1 levels. In addition to typical findings of pancreatic carcinoma, a solid mass was observed in the spleen by imaging procedures. On ultrasonography, the splenic mass was well demarcated and slightly hypoechoic. Computed tomography demonstrated a homogeneous low-density mass 5 cm in diameter. On T1- and T2-weighted magnetic resonance images, the splenic mass was demonstrated as low intensity and high intensity, respectively. On selective angiography, the tumor was hypervascular. Distal pancreatectomy plus splenectomy was performed. Microscopically, the splenic tumor consisted of red pulp tissue and was diagnosed as splenic hamartoma.     

Hemoclip-Assisted Polypectomy of Large Duodenal Brunner's Gland Hamartoma

Brunner's gland hamartoma, also known as Brunneroma or Brunner's gland adenoma, is a rare tumor of the duodenum and an uncommon cause of gastrointestinal bleeding. In symptomatic patients, treatment requires either surgical resection or endoscopic polypectomy. We report a case of upper gastrointestinal bleeding from a pedunculated Brunner's gland hamartoma in the duodenal bulb. A new technique using hemoclip-assisted polypectomy is described to remove a large pedunculated Brunner's gland hamartoma.     

Spontaneous rupture of splenic hamartoma in a patient with hepatitis C virus-related cirrhosis and portal hypertension： A case report and review of the literature

Spontaneous rupture is a rare complication of splenic hamartoma. A review of the literature revealed only four such cases. To the best of our knowledge, this is the first report of spontaneous rupture of splenic hamartoma associated with liver cirrhosis and portal hypertension. A 53-year-old woman, who was followed up for aortic dissection and hepatitis C virus (HCV)-related liver cirrhosis, was referred with sudden left chest and shoulder pain. An abdominal ultrasound showed intraabdominal bleeding, and computed tomography indicated rupture of a splenic tumor. Emergent splenectomy was carried out. The postoperative course was uneventful, and the patient was discharged on the 13th postoperative day. Pathology revealed the tumor to be a ruptured splenic hamartoma. The non-tumorous splenic parenchyma revealed congestive changes. We consider that the presence of liver cirrhosis and portal hypertension are risk factors for spontaneous rupture of the splenic hamartoma.     

Differentiation of a rat mammary cell line in vitro.

We have studied the development of fusiform (probably related to myoepithelial) cells in Rama 25 cultures [Bennett, D. C. Peachey, L. A., Durbin, H. & Rudland, P. S. (1978) Cell 15, 283--298]; we show that they are generated from special differentiated structures (projections) that contain a rapidly differentiating cell type (F-precursor cells). Clonal sublines isolated from projections develop in several directions under both environmental and genetic control. Some types of differentiation are reversible; others are irreversible. The various cell occurring in vitro may correspond to specific cell types in vivo.     

Myoepithelial carcinoma of the stomach: A diagnostic pitfall

Myoepithelioma/myoepithelial carcinomas are not commonly found in soft tissues and are especially rare at visceral sites. This report describes a case of a rare low-grade myoepithelial carcinoma of the stomach. A61-year-old female patient presented with postprandial abdominal discomfort. Endoscopy revealed a 1.1 cm submucosal lesion. Local excision was performed after malignancy was confirmed by biopsy. The resection margin is free of tumor and she received no adjuvant therapy. The tumor was characterized by multinodular growth with biphasic epithelioid and spindle components. Infiltrative margin and nuclear pleomorphism are seen. Tumor cells were positive for both epithelial and myoepithelial markers. Evidence of epithelial differentiation was confirmed by electron microscopy. No EWSR1 rearrangement was detected. The final diagnosis was low-grade myoepithelial gastric carcinoma. The patient is currently well, and no evidence of recurrence or metastasis was found after ten-month of follow-up. Myoepithelial carcinoma should be considered in the differential diagnosis of a biphasic gastric tumor.