Adult ; Female ; Hamartoma ; complications ; pathology ; Humans ; Peutz-Jeghers Syndrome ; complications ; pathology ; Polyps ; complications ; pathology

Lipofibromatous hamartoma of the nerve is a benign tumor, which affects the major nerves and their branches in the human body. It is often found in the median nerve of the hand and is commonly associated with macrodactyly, but it is rarely found in the digital nerves at the peripheral level. This tumor is often found in young adults and may go through a self- limiting course. However, operation is indicated when the tumor size is large or when the associated nerve compressive symptoms are present. We have experienced a rare case of lipofibromatous hamartoma that symmetrically involved the volar digital nerves of both index fingers on the ulnar side. With the aid of a microscope, we dissected and removed the tumor as much as possible without sacrificing the nerve. No sensory change occurred in both fingers and no sign of recurrence was observed upon follow-up.
Adipose Tissue/pathology ; Adult ; Female ; Fingers/*innervation/pathology ; Hamartoma/complications/*pathology ; Humans ; Nerve Compression Syndromes/etiology/*pathology

Adult ; Female ; Hamartoma ; complications ; diagnosis ; surgery ; Humans ; Kidney Calculi ; complications ; Spleen ; pathology ; surgery ; Splenectomy ; methods ; Splenic Diseases ; complications ; diagnosis ; surgery

Cowden's disease, also known as various hamartomatous malformations of multiple organs, is a rare autosomal dominant disorder. The most important feature of Cowden's disease is its frequent association with malignant neoplasm, particularly in the breast and thyroid gland. Cowden's disease with malignant neoplasms, is quite rare in Korea so far. We report a case of Cowden's disease associated with breast cancer in a 41-year-old female who underwent routine health cheek-up.
Adult ; Breast Neoplasms/*complications/pathology ; Colonoscopy ; Female ; Gastrointestinal Tract/pathology ; Gastroscopy ; Hamartoma Syndrome, Multiple/complications/*diagnosis ; Humans ; Polyps/diagnosis

〓 Objectives: To analyze the pathological and clinical features of nasal respiratory epithelial adenomatoid hamartoma（REAH）, and summarize the diagnostic points, to improve the experience of diagnosis and treatment. Methods:The clinical data of 16 patients with REAH were analyzed retrospectively. The clinical manifestations, pathological features, imaging features, surgical treatment and prognosis were summarized. Results:16 cases of REAH were studied, 10 cases（62.50%） were associated with sinusitis, 1 case（6.25%） was associated with inverted papilloma, 1 case（6.25%） was associated with hemangioma. 5 cases（31.25%） had a history of nasal sinus surgery, including 1 case with 3 times of nasal sinus surgery, 1 case with 2 times of nasal sinus surgery, 3 cases with 1 time of nasal sinus surgery; 10 cases（62.50%） occurred in the bilateral olfactory cleft, 2 cases（12.50%） in the unilateral olfactory cleft, 3 cases（18.75%） in the unilateral middle turbinate, 1 case（6.25%） in the nasopharynx. All 16 patients were pathologically diagnosed as REAH. In the patients with lesions located in bilateral olfactory fissures, symmetrical widening of olfactory fissures and lateral displacement of middle turbinate were observed on preoperative sinus CT. The average width of bilateral olfactory fissures was （9.9±2.70） mm. The ratio of wide to narrow olfactory cleft was 1.21 ± 0.19. There was no significant difference in Lund-Mackay score between the two sides（P>0.05）. All patients underwent surgery under general anesthesia and nasal endoscopy. The follow-up period ranged from 1 to 66 months, and no recurrence occurred. Conclusion:Preoperative diagnosis of REAH is facilitated by the combination of clinical manifestations and endoscopic and imaging features. Endoscopic complete resection can achieve a good therapeutic effect.
Humans ; Nasal Polyps/complications* ; Retrospective Studies ; Paranasal Sinuses/pathology* ; Adenoma ; Endoscopy/methods* ; Hamartoma/surgery*

OBJECTIVETo analyze the histologic characteristics of macrochilia secondary to port-wine stain and to elucidate the possible mechanism.

METHODSTwenty-one cases of macrochilia secondary to venular malformation were included and the histology of the lesions was observed by light microscope.

RESULTSHistological examination revealed vascular abnormalities and a number of widely distributed hamartomatous changes in macrochilia secondary to venular malformation. The average vessel diameter is (39.8 +/- 15.7) microm. The degree of hamartomatous change: mild (1 case), moderate (7 cases) and severe (13 cases).

CONCLUSIONSThe complex hamartomatous changes suggest a genetically determined, multilineage developmental field defect in the pathogenesis of venular malformation.

Adolescent ; Adult ; Female ; Hamartoma ; etiology ; pathology ; Humans ; Lip ; abnormalities ; pathology ; Lip Diseases ; etiology ; pathology ; Male ; Microvessels ; pathology ; Middle Aged ; Port-Wine Stain ; complications ; pathology ; Young Adult

The clinical data for a patient with primary lung adenocarcinoma complicated with pulmonary hamartoma, who admitted to Zunyi Medical University Hospital in September 2020, was retrospectively analyzed. The 62-years-old male visited outpatient service because of dysphagia in March 2015, and the pulmonary nodules were found. In September 2020, the computed tomography indicated the enlarged nodule in the lower lobe of left lung with lobulation, and there was ground glass nodule in the upper lobe of left lung. After thoracoscopic wedge surgery, the primary pulmonary adenocarcinoma in the upper lobe of left lung and pulmonary hamartoma in the lower lobe of left lung were confirmed by pathology. Whole exon sequencing revealed that kinesin family member 20B (KIF20B) gene was not expressed in lung adenocarcinoma, but was expressed in pulmonary hamartoma. The clinical manifestations of lung adenocarcinoma complicated with pulmonary hamartoma was not typical, which could locate in the same side and different sides of the lung. The imaging manifestations of the 2 kinds of tumors were diverse and can not be completely distinguished. The pathological examination after surgery is the gold standard, and the possibility of malignant transformation of pulmonary hamartoma should be warned.
Adenocarcinoma of Lung/complications* ; Hamartoma/surgery* ; Humans ; Kinesins ; Lung/pathology* ; Lung Neoplasms/surgery* ; Male ; Middle Aged ; Retrospective Studies

No abstract available.
Aged ; Bile Duct Neoplasms/etiology/*pathology ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma/etiology/*pathology ; Hamartoma/complications/pathology ; Humans ; Immunohistochemistry ; Liver/pathology/surgery ; Male ; Tomography, X-Ray Computed

A 5-year-old girl was diagnosed with neurofibromatosis type 2 (NF-2) due to multiple neurofibromas, cafe-au-lait spots, and schwannomas of the brain. During ophthalmologic evaluation, a posterior subcapsular cataract and a gray-green colored subretinal lesion were found in right eye. Fluorescein angiography (FA) revealed a combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). At age 9, she underwent cataract surgery. At this time FA and spectral-domain optical coherence tomography (SD-OCT) were taken. The SD-OCT showed an elevated hyperreflective mass in the retina with prominent attenuation of the inner and outer retina, but minimal attenuation in the photoreceptor layers. The underlying retina appeared to be disorganized and thick (791 microm). This is the first case report of SD-OCT imaging of a CHRRPE associated with NF-2 in a pediatric patient. By using SD-OCT in this patient, we could obtain detailed tumor characteristics, and SD-OCT may be helpful in the diagnosis and management of CHRRPE.
Child, Preschool ; Diagnosis, Differential ; Female ; Hamartoma/complications/*diagnosis ; Humans ; Neurofibromatosis 2/*complications/diagnosis ; Retinal Diseases/complications/*diagnosis ; Retinal Pigment Epithelium/*pathology ; Tomography, Optical Coherence/*methods ; Visual Acuity

OBJECTIVETo study clinical and imaging features of hypothalamic hamartoma in children.

METHODSImaging findings and clinical manifestations of 38 children with hypothalamic hamartomas were retrospectively reviewed. The patients included 25 boys and 13 girls, ranging in age of onset from 1 month to 15 years. All the 38 patients were examined with pre-contrast and post-contrast T1 weighted MR imaging and with non-contrast T2 weighted MR imaging. Meanwhile, 10 patients received CT scan. Hypothalamic hamartomas were confimed by pathologic examinations in the 38 patients.

RESULTSGelastic epilepsy, precocious puberty, other types of epilepsy and disturbance of intelligence were main manifestations in the 38 patients. Gelastic epilepsy was found as an initial clinical symptom in 17 cases, precocious puberty in 13 cases and other types of epilepsy in 8 cases. All masses were located in the suprasellar and interpeduncular cistern. They showed iso-density on CT scan, and homogeneous signal iso-intense to gray matter on T1 and T2 weighted MR images. With contrast MR images, there was no enhancement in the mass. The size or pedunculation of the mass was not correlated with clinical features.

CONCLUSIONSHypothalamic hamartoma may be characterized by precocious puberty and/or gelastic epilepsy and specific imaging findings mentioned above.

Adolescent ; Adult ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Hamartoma ; complications ; diagnosis ; pathology ; Humans ; Hypothalamic Neoplasms ; complications ; diagnosis ; pathology ; Infant ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed