Primary mesenchymal liver tumors of childhood

Primary mesenchymal tumors of the liver represent a significant proportion of liver tumors among neonates, infants and young children. They may be discovered incidentally or present with an alarmingly large, rapidly growing abdominal mass. One third of pediatric liver tumors are benign with hemangioma and mesenchymal hamartoma being the commonest in that order. Infantile hemangioendothelioma and mesenchymal hamartoma represent the commonest liver tumors among neonates and infants. Mesenchymal hamartoma may even present in utero. A biopsy is often required for definitive diagnosis due to considerable overlap in clinical presentation and radiological findings amongst these tumors, as well as with hepatoblastoma.     

Mesenchymal hamartoma of the liver--a case report

Mesenchymal hamartoma of the liver is a rare benign tumor of infancy and childhood. A case of mesenchymal hamartoma of liver seen in a 37 day old child is reported and the available literature on the subject is briefly reviewed.     

Role of fine-needle aspiration in clinical management of transplant patients

Fine-needle aspiration (FNA) of superficial and deep seated lesions has been used with high sensitivity and specificity in the diagnosis of neoplastic and non-neoplastic entities. However, studies of FNA in post-transplant patients are virtually absent. Six hundred and seventy-four allograft recipients (cardiac 288, renal 250, lung 131 and heart-lung 5) were reviewed. A total of 30 (25 heart, 4 lungs and 1 renal transplant) patients underwent an FNA procedure. There were 26 males and 4 females. Ages ranged from 18–63 yr (mean 48 yr). The most common entity aspirated was post-transplant lymphoproliferative disorder (PTLD) in 12 cases, followed by inflammatory lesions in 10 cases, malignant epithelial neoplasms in 3 cases, and 1 case each of malignant mesenchymal tumor, pulmonary infarction, hamartoma of liver, fatty changes of liver, and a benign vascular lesion. Surgical or autopsy tissue was available in 19 cases (63.3%). There was an agreement between tissue diagnosis and FNA material in 18 cases (94.7%). One (5.2%) false negative case was recorded. This was a liver aspirate showing benign liver elements, which a surgical biopsy proved to be a bile duct hamartoma. No false positive cases were recorded. FNA is a highly sensitive and specific diagnostic tool in the management of post-transplant patients. Diagn. Cytopathol. 17;1997:429–435. © 1997 Wiley-Liss, Inc.     

Mesenchymal hamartoma of liver in a man: comparison with cases in infants.

A 53 year old man with a large mesenchymal hamartoma is reported. Only a few bile ducts could be found in the periphery of the lesion and no hepatocytes were identified within the lesion. As far as is known, this is the only adult male patient reported to date. On the basis of the reported findings of mesenchymal hamartoma in other adults, it is suggested that there could be changes in the morphology of this lesion with age: progressive loss of hepatocytes; degeneration of bile duct epithelium; and cystic change of the mesenchymal component. The haematopoietic element is considered to be part of the fetal hepatic haematopoiesis that occurs in the hamartoma.     

Mesenchymal hamartoma of the chest wall in an infant

Mesenchymal hamartoma of the chest wall is a very rare, benign tumour with distinct clinical, radiological and histopathologic characteristics. The lesion develops during foetal life, and is present at or shortly after birth with an extrapleural mass arising from the rib cage with or without respiratory distress and marked rib deformity. Several imaging techniques have been used for diagnosis, but a definitive diagnosis is established only by histopathological examination. Such lesions are composed of a varying admixture of hyaline cartilage that has features resembling growth plate cartilage, along with fascicles of spindle cells, woven bone and hemorrhagic cysts. Accurate diagnosis of mesenchymal hamartoma is important since many chest wall masses in children are malignant. We report a case of mesenchymal hamartoma of the left posterior chest wall surgically resected in an infant who was found to have a palpable mass at birth. Two years after surgery, the patient is alive and well, with no evidence of recurrence.     

Diffuse pulmonary hamartoma: a case report

Pulmonary hamartoma usually occurs as a benign, well-circumscribed single nodule in the lung parenchyma. We report on a unique case of hamartoma that extended along the bronchial tree, formed endobronchial polypoid lesions, and expanded into the lung parenchyma. The patient, a 48-year-old man, was admitted with dyspnea and chest pain. A transbronchial biopsy was performed on a tumorous lesion that was diagnosed histologically as a hamartoma. As this lesion was found to be growing diffusely along the bronchial tree, a left pneumonectomy was performed. Gross examination showed that yellowish soft tissue had surrounded the bronchial tree and extended into the lung parenchyma. Histologically, the lesion contained mainly mature adipose tissue, cartilage, and muscle tissue with a minor component of short spindle cells in a myxomatous matrix. The patient was diagnosed as having diffuse pulmonary hamartoma.     

Mammary hamartoma--a case report

Mammary hamartoma is a rare lesion commonly associated with Cowden syndrome. It presents clinically as a fibroadenoma. The nonspecific cytological findings makes the pre-operative diagnosis of this lesion difficult. Though hamartomas of the breast are predominantly benign, co-existent malignancies within them have been reported. These lesions also have a tendency to recur; hence a correct diagnosis and extensive sampling of hamartoma is essential. We report a case of mammary hamartoma with a brief review of literature.     

Case of fibrolipomatous hamartoma of the digital nerve without macrodactyly

Fibrolipomatous hamartoma of nerves without macrodactyly is a rare lesion characterized by fibrofatty proliferation causing epineural and perineural fibrosis with fatty infiltration around the nerve bundles. We report an unusual case of fibromatous hamartoma of the ulnar digital nerve of the thumb in a 43-year-old woman. Magnetic resonance imaging revealed a large fusiform mass along the nerve. The findings were unusual and pathognomonic and included a coaxial cable-like appearance on axial sections and a spaghettilike appearance on coronal sections on both T1- and T2-weighted images; these findings were useful for the diagnosis and preoperative evaluation of this lesion. Surgical exploration revealed a yellow, cordlike mass of the digital nerve enlarged by fat. Gross excision could not be done without extensive damage to the nerve. Therefore, a limited excision with biopsy of the fibrolipomatous tissue around the nerve bundles was performed. The histological appearance was consistent with fibrolipomatous hamartoma. There was no recurrence of the mass and no neurological deficit 3 years after surgery. Some authors have suggested that invasive excision can cause catastrophic sensory or motor deficits because of the extensive fatty infiltration of the nerve fascicles. In conclusion, the recommended treatment for this lesion is limited excision with only biopsy to confirm the diagnosis.     

Pleomorphic fibrohistiocytoma of the breast: a potential pitfall in breast biopsy interpretation

We describe a benign mammary mesenchymal tumour with atypical stromal giant cells in the contralateral breast of a 66-year-old woman with infiltrating ductal carcinoma. The clinical, morphological and immunohistochemical features of this tumour suggest a pleomorphic variant of fibrous histiocytoma. This benign lesion represents a possible pitfall in breast pathology when interpreting a frozen section or fine needle aspiration biopsy.     

Hepatic mesenchymal hamartoma: a short review

Hepatic mesenchymal hamartoma is a hamartomatous growth of mesenchymal tissue in the liver of uncertain etiology. It is a space-occupying lesion that can potentially compress adjacent organs resulting in various complications including death. Hepatic mesenchymal hamartoma is characterized by proliferation of variably myxomatous mesenchyme and malformed bile ducts. The differential diagnosis includes other pediatric hepatic masses. The diagnosis is typically made during infancy, and complete resection is invariably curative.     

Correlation of digital rectal examination, prostate specific antigen, and transrectal ultrasound in prostate carcinoma in African Americans.

Since there is general agreement that screening for prostate cancer should be carried out, at least for high-risk individuals, there should be little debate that African-American men should be screened. Current screening guidelines include the two most cost-effective methods of early detection, digital rectal examination and prostate specific antigen. The use of transrectal ultrasound and guided biopsy improves the yield. This article reports on the findings of 50 African-American patients with prostatic carcinoma diagnosed by sonographically guided biopsy in a single, community urology practice. Overall, prostate specific antigen was elevated in 94%, digital rectal examination was positive in 60%, and transrectal ultrasound was positive in 78%. A focal hypoechoic lesion was demonstrated in 58%. When the site of tumor, as specified in the pathology report, was correlated with the findings on digital rectal examination and transrectal ultrasound, both digital rectal examination and transrectal ultrasound were positive in 45%. Transrectal ultrasound was positive when digital rectal examination was negative in 30%. Digital rectal examination was positive when ultrasound was not in 14%. Random biopsy revealed areas of carcinoma that were not detected by digital rectal examination nor ultrasound in 40%. We conclude that even though random biopsy significantly improves the detection of prostate carcinoma, sonographic guidance is beneficial to systematically biopsy the gland and to avoid omission of characteristic lesions during random samplings.     

Optical imaging as an adjunct to sonograph in differentiating benign from malignant breast lesions

The role of near infrared (NIR) diffusive light imaging as an adjunct to ultrasound in differentiating benign from malignant lesions was evaluated in 27 mammography patients with infiltrating ductal carcinomas, apocrine metaplasia, fibroadenomas, radial scar and ductal hyperplasia, cysts, and normal tissues. Conventional ultrasound/mammography images were graded based on BI-RADS assessment categories. The spatial NIR measurements were made at wavelengths of 750 and 830 nm. Functional images, such as relative changes of deoxyhemoglobin (deoxyHb) and total blood concentration, were estimated from the dual wavelength measurements. Maximum relative deoxyHb and blood concentration changes were measured, and spatial correlation of masses in relative deoxyHb and blood concentration images for each breast were calculated. For the five biopsy proven benign lesions, ultrasound/mammography diagnoses were suspicious for malignancy (four cases) and highly suspicious for malignancy (one case). Four lesions showed less than 1.0 V maximum deoxyHb and less than 1.5 V maximum blood concentration levels on average and spatial image correlation showed no correlated masses in both deoxyHb and blood concentration images. For the four biopsy proven malignant lesions, ultrasound/mammography diagnoses were highly suspicious for malignancy. Maximum deoxyHb and blood concentration changes were greater than 2.9 V on average except one lesion which showed smaller deoxyHb signal (maximum 0.85 V) but the deoxyHb mass and blood concentration mass were highly correlated.     

不同肝段超声引导下穿刺活检的临床分析

目的 总结不同肝段超声引导下穿刺活检的经验及体会。方法 回顾性分析我院于2015年3月~2018年7月收治入院肝脏疾病患者60例,分析不同肝段疾病在超声引导下穿刺活检术临床资料,总结超声引导下穿刺活检术在提高肝脏疾病诊断的临床价值及病理取材操作准确性的心得。结果 本组共60例病例,其中Ⅱ段16例、Ⅲ段8例、Ⅳ段4例、Ⅴ段5例、Ⅵ段7例、Ⅶ段9例、Ⅷ段11例,出现1例并发症（出血）。60例病例中弥漫性病变28例:其中肝硬化11例、肝炎17例;良性病变18例:其中血管瘤13例、肝脓肿5例;恶性肿瘤14例     

Lymphangiomyoma

A case report is presented of the incidental finding of a lymphangiomyoma in a 50-year-old woman who underwent total hysterectomy for squamous cell carcinoma of the cervix. The condition seems to occur only in women after puberty and so far, has been mainly reported in association with chylothorax or chylous ascites. There is no evidence of any clinical complication more than 2 years after removal of the lesion. The lesion is most likely a hamartoma although the term lymphangiomyoma is acceptable. The term lymphangiomyomatosis is best reserved for the fully developed clinico-pathological syndrome.     

Squamous cell carcinoma arising in a ciliated hepatic foregut cyst

We report a case of squamous-cell carcinoma arising in a ciliated hepatic foregut cyst that occurred in a 21-year-old man. The cystic lesion was first discovered during childhood with no further follow-up. Following important weight loss over several months, the patient was admitted to our hospital where a CT scan showed a cystic and solid mass in segments V and VI of the liver involving the transverse mesocolon and the gastric antrum. A right hepatectomy with en-bloc right hemicolectomy and partial gastrectomy was performed. Gross examination showed a partially cystic liver mass with a maximum dimension of 10 cm infiltrating the large bowel wall. Microscopically, it was a poorly differentiated squamous-cell carcinoma arising from the wall of a liver cyst lined by a ciliated, pseudostratified columnar epithelium. Hepatic foregut cysts are uncommon, congenital, benign lesions that, when discovered, deserve careful clinical follow-up as malignant transformation, albeit exceptional, is possible.     

Malignant mesenchymoma of the liver in children: A clinicopathologic and ultrastructural study

A primary malignant mesenchymona of the liver in a nine year old boy is reported with the results of ultrastructural investigation. In accordance with previous observations, our study indicates that this type of tumor is remarkable because of its bulky size and possible rapidly fatal outcome. Although the term malignant mesenchymoma can be applied, the tumor is primarily fibrosarcomatous and liposarcomatous. It could be postulated that this tumor and the more frequent benign mesenchymal hamartoma of the liver are two histogenetically related lesions that preferentially affect the liver in children.     

Solid mesenchymal hamartoma of the liver in adult.

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.     

Ductal carcinoma in situ arising in mammary hamartoma

Hamartoma of the breast is an uncommon lesion. Although it can possess characteristic radiological features, the pathological appearance is not distinctive. Hamartoma is generally considered benign, but four cases have been reported with ductal and lobular carcinoma arising in hamartomas. This report describes further cases of hamartoma from which ductal carcinoma in situ arose, with one showing early invasion. In both cases, the tumours were within the hamartomas and were adequately excised during lumpectomies of the hamartomas, and the patients were well afterwards. This report emphasises the importance of adequate sampling of mammary hamartoma.