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PubMed2008

MRI findings in Shwachman diamond syndrome.

Shwachman Diamond Syndrome (SDS) is a rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction, and skeletal changes. Because of the heterogeneous clinical presentation and the limits of laboratory tests that assess pancreatic insufficiency, the diagnosis of SDS can be challenging. Pancreatic lipomatosis, a typical feature of this syndrome, is also difficult to assess by direct tissue sampling. In these circumstances, magnetic resonance imaging (MRI) provides a readily available, noninvasive tool to evaluate the pancreatic fat content. We report a case of a 12-month-old male in which abdominal MRI was used to confirm the clinical diagnosis of SDS.

Bone Marrow Diseases↗
PubMed1982

Hypoxic alterations of tenocytes in degenerative tendinopathy.

The authors examined 34 tendons under the electron microscope, excised within 48 h after rupture of the tendons. The tendons did not exhibit any signs of inflammation or lipomatosis, but fine-structurally marked hypoxic alterations in the tenocytes could be seen. The degenerative (hypoxic tendinopathy appears in three phases, and the hypoxic lesions of the tendon occur at multiple foci or diffusely, and the various phases can exist simultaneously.

Adolescent↗
PubMed1978

[Pathohistological findings in infarcted parotid glands (necrotizing sialometaplasia) (author's transl)].

In 1973 Abrams et al. described a nonneoplastic lesion named "necrotizing sialometaplasia". Since this date additional cases have been reported. The lesion might be misdiagnosed either mucoid tumor or squamous carcinoma. The cases reported in the following years were localised on the hard palate, lower lip, nose and in the major salivary glands. By the "Speicheldrüsenregister" of the Institute of Pathology of the University of Hamburg five cases of infarcted parotid glands were registered during the years of 1965--1977. All infarcts are observed in patients with tumors in the parotid region. No infarct happened spontaneously. Histologically two forms of infarcts are distinguished: haemorrhagic and ischaemic. In both forms of infarcts there necrosis of acini and squamous metaplasia in intercalar and striated ducts. Degenerative changes are seen in the center of ducts with squamous metaplasia. The reaction of the interstices of the two forms of infarction are different: In the ischaemic there lipomatosis, and in the haemorrhagic there are fibrosis. Our findings are exactly as described for minor salivary glands in necrotizing sialometaplasia.

Humans↗
PubMed1988

Axillary lymph node morphology in women with in situ breast carcinoma. An autopsy study.

Axillary lymph nodes in 184 female autopsy cases were studied using morphological criteria. Special attention was paid to the morphology in 34 women with in situ breast carcinoma (in situ BC) compared to the remaining women without malignant breast lesions, who served as controls. Sinus histiocytosis (SH) and diffuse cortical hyperplasia (DCH) were significantly more frequent among women with in situ BC compared to controls. No significant association was found between unilateral in situ BC and these lymph node patterns on the contralateral side. Germinal center and follicular hyperplasia (GCH/FH), lymphocyte depletion (LD), fibrosis, hyalinization, calcifications and lipomatosis were not associated with in situ BC. The results indicate that in situ BC provokes reactive morphological changes of the regional axillary lymph nodes similar to the changes associated with a good prognosis in women with invasive breast cancer (IBC).

Adult↗
PubMed1986

Computed tomography in the differential diagnosis of the enlarged retrorectal space.

The value of computed tomography (CT) in the differentiation of an enlarged retrorectal space was analyzed in 132 cases. Classification of barium enema findings into those with simultaneous mucosal alterations and those without any visible lesions of the rectal mucosa seems to be useful. Computed tomography helps in those cases without mucosal changes to differentiate between retrorectal fibrosis, tumorous masses, and inflammatory diseases of the colon. It also demonstrates the lack of pathologic lesions in equivocal cases of pelvic lipomatosis and so-called "normal variants." If simultaneous mucosal involvement on barium enema--especially in rectal carcinoma or recurrent carcinoma of the rectum--is found, CT may show the perirectal extension of tumorous masses and thus help to clarify local operability.

Abscess↗
PubMed1985

Myocarditis in autopsy.

In the Annuals of Autopsy Records for Japan from 1958 to 1977, 377,841 autopsy cases were registered; a short summary of pathologic findings was included. Of the 377,841 cases, 409 (0.11%) were registered as idiopathic, nonspecific, interstitial, or viral myocarditis and 25 cases (0.007%) as giant-cell myocarditis. The annual incidence of the myocarditis varied at 5-year intervals and a remarkable increase was observed after 1974. An analysis of 19 cases of myocarditis and 19 of postmyocarditic cardiomegaly (PMC) showed a preponderance of males. Dilatation of ventricles was found in all cases of acute, subacute, and healing myocarditis, which histologically showed interstitial mononuclear cell infiltration, necrosis, and disarray of myocytes. Hypertrophy with ventricular dilatation, ventricular dilatation without hypertrophy, endo- or epicardial involvement, and right ventricular lipomatosis were found in 70%, 20%, 30%, and 10% of cases with chronic myocarditis and PMC, respectively. In these cases, residual inflammation, fibrosis, and hypertrophy was observed histologically.

Adolescent↗
PubMed1991

Functional applications of suction-assisted lipectomy: a new treatment for old disorders.

Ever since the introduction of suction-assisted lipectomy in the United States in 1981, the technique has been applied to an increasing number of disorders of the subcutaneous tissues. Indeed, suction-assisted lipectomy has evolved into the method of choice in the treatment of certain pathological entities. While the extraction of lipomas is the most common functional application, suction-assisted lipectomy has also been used successfully to treat such conditions as gynecomastia, axillary hyperhidrosis, benign symmetric lipomatosis (Madelung's disease), congenital body asymmetry, congenital or acquired lymphedema, flap defatting, traumatic or postoperative hematomas, and fat necrosis. We have successfully treated patients presenting with the above disorders. No morbidities or mortalities were encountered in our series of 18 patients. The final results were considered favorable by the majority of patients. Acceptance by the patients of this treatment modality was extremely high because of the smaller incisions required, the exactness in contouring, the simple and minimal postoperative care needed, and the ease with which the procedure can be repeated to refine the results. A comprehensive review of the literature is presented along with our own patient management and long-term results.

Adult↗
PubMed1987

Colonic lipomas. Report of two unusual cases and review of the Mayo Clinic experience, 1976-1985.

Two cases of symptomatic submucosal lipomas of the large intestine are described. One occurred in a patient with familial multiple lipomatosis causing an intussusception and intermittent obstruction. The other caused subacute obstruction and rectal bleeding and was thought to represent a carcinoma. These two cases closely resemble those few patients with symptomatic lipomas of the colon that make up a small subgroup (6 percent) of a series of 91 patients with this diagnosis managed surgically at the Mayo Clinic between the years 1976 to 1985. The majority of patients in this series had lipomas that were entirely incidental findings usually associated with more significant pathology that dictated the operative procedures undertaken. Lipomas themselves may be managed by local excision only although segmental resection may be necessary in isolated cases.

Aged↗
PubMed1988

Nonenzymatic isolation and culture of adult islets from atrophic pancreata of copper-deficient rats: a morphologic analysis.

The purpose of this study was to develop a nonenzymatic method of isolating adult islets using atrophied pancreata from copper-deficient rats and to analyze their morphologic characteristics and behavior in culture. This unusual model of isolation was studied because islets remain intact in the course of dietary copper deficiency while the acinar glandular component of the pancreas undergoes selective atrophy and lipomatosis. Small fragments containing islets were readily microdissected from atrophied glands and placed in culture. Within 24 h the fragments congealed into small irregular- to spherical-shaped masses within which the darker profile of islets could be distinguished. Within a period of 3 to 5 d, islet tissue began to bud from the lipocytic mass until by Day 7 spherical aggregates of intact islet tissue separated from the residual fragments. Subsequent to further in vitro treatment, these islets could be maintained as free viable spherical masses if periodically agitated, as attached stationary islets which developed monolayer growth if left undisturbed and as aggregated masses of islet tissue forming megaislets if combined in small groups. Grouped islets treated with actinomycin D and cycloheximide did not exhibit aggregation when incubated with these inhibitors. This suggests that megaislet formation was an active process requiring protein-RNA synthesis rather than passive clumping or aggregation that can accompany metabolically altered or dying islets undergoing cellular shedding and adhesion. Immunohistochemical localization demonstrated that insulin, glucagon, somatostatin, and pancreatic polypeptide-immunoreactive cell types were present within the islets derived from this technique. The cellular topography of these islets was not unlike that described by others for islets cultured from enzymatic isolation. This culture model may serve as a resource for mature, viable islets isolated without mechanical or enzymatic disaggregation which can have attenuating effects on islet function.

Animals↗
PubMed2002

Islet hyperplasia in callitrichids.

Five callitrichids (three common marmosets -Callithrix jacchus -, a black tufted-eared marmoset -C. penicillata-, and a saddle-back tamarin -Saguinus fuscicollis) were diagnosed with islet hyperplasia by histopathology and immunohistochemistry. All were privately-owned, unrelated callitrichids ranging from 2- to 4-year-old. Relevant findings were anorexia (3/5), vomiting (2/5), ptyalism (1/5), polyuria/polydipsia (1/5), respiratory distress (1/5), hyperglycemia (2/3) and glycosuria (1/1); hyperglycemia and glycosuria were associated with pregnancy in a common marmoset and resolved after reducing simple carbohydrates in diet. All five animals died, three of them after few premonitory signs; in two cases, other concurrent diseases unrelated to islet hyperplasia were considered the cause of death. Additional animals from two facilities had high weight (4), physical obesity (3), polyuria/polydipsia/polyphagia/uriposia (1), hyperglycemia (1), and/or glycosuria (2). Pathologic findings in the deceased callitrichids were: islet hyperplasia (5/5); hemosiderosis (5/5); lipomatosis (4/5) of several tissues (atria, 3/5; pancreas, gall bladder, intestine, esophagus, and thyroid, 2/5; liver, 1/5); pancreatic necrosis or steatonecrosis, and/or acute pancreatitis (3/5); and vacuolation of hepatocytes and renal tubular cells most likely consistent with hepatorenal lipidosis (2/5). The islets of Langerhans were more numerous and larger than in a control, and morphologically normal in all cases, except in a common marmoset that had a few cells with a foamy cytoplasm and shrunken hyperchromatic or picknotic nucleus. Insulin (5/5), glucagon (3/5), and somatostatin (3/5) immunohistochemistry revealed that most cells stained positively for insulin diffusely in their cytoplasm (5/5) (staining restricted to the vascular pole of b-cells in the control). These findings suggest that obesity, insulin resistance and/or type II diabetes may be implicated and thus a prospective study on these diseases in callitrichids is necessary to determine their etiopathogenesis.

Animals↗
PubMed2003

Congenital fibrolipoma of anal canal.

Although rare, congenital lipomatosis presents during first few months of life as large sub-cutaneous fatty masses on chest with extension into skeletal muscle bundles. Only a few such cases have been reported in the literature. A rare case of congenital fibrolipoma of anal canal in a 3-day-old male child is being documented in the present report.

Anal Canal↗
PubMed2006

Hemihyperplasia syndromes.

OBJECTIVE: Hemihyperplasia is a heterogenous group of disorders characterized by asymmetric limb growth. There is considerable confusion regarding their classification and ascertainment into various syndromes. We tried to look into the various aspects of hemihyperplasia syndromes. METHODS: Records of 17 consecutive cases of hemihyperplasia were reviewed and were ascertained into various syndromes based on available literature and diagnostic criteria. RESULTS: Of the 17 cases with hemihyperplasia, 3 cases satisfied the diagnostic criteria for Proteus syndrome. One patient each was ascertained as Klippel Trenaunay Weber syndrome and Hemihyperplasia- Multiple lipomatosis. 9 cases were classified as isolated hemihyperplasia. We found two novel associations with hemihyperplasia; namely Ehlers-Danlos syndrome like skin changes and Poland anomaly on the affected side. The remaining 3 cases had miscellaneous disorders with limb asymmetry, namely Neurofibromatosis Type I in 2 cases and Olliers disease in one case. CONCLUSION: Efforts to diagnose syndromes of hemihyperplasia help in genetic counseling.

Adolescent↗
PubMed1989

Progressive changes in pancreatic vasculature accompanying copper deficiency-induced glandular atrophy.

Pancreatic vasculature was studied in rats treated with a dietary copper-deficient regimen to assess and characterize changes in the exocrine angioarchitecture of the gland. The deficient state is known to induce progressive acinar degeneration, atrophy, and subsequent replacement with noninflammatory lipomatosis. This study was carried out since little information exists regarding changes in the vasculature that accompany acute glandular necrosis. Corrosion casts produced from Mercox injected deficient rats were studied using scanning electron microscopy and compared to injected preparations studied by light microscopy. Results show that with initial atrophy of degenerative acini (6-8 wk), pancreatic lobules decreased in size and the volume of the vascular bed reduced. From wk 8 onward, the accumulation of fat cells expanded the configuration of lobules to more normal dimensions. Although vascular interconnectivity within lobules remained reduced, its basic angioarchitecture remained intact and served to vascularize fat cells in lieu of acini. No acute angiopathic changes indicative of vascular disorganization were found. These observations support the hypothesis that the vasculature of the atrophied pancreas, although modified in regions of the gland most severely targeted by acinar necrosis, remains intact and its basic structural features preserved. Preliminary evidence also supports the observation that preservation of the basic elements of lobular angioarchitecture may form the basis for subsequent regeneration of acinar tissue in the reversed deficient state.

Animals↗
PubMedDate not supplied

The use of suction-assisted surgical extraction of moderate and large lipomas: long-term follow-up.

Solitary lipomas and familial multiple lipomatosis are the most common benign tumors and are very well encapsulated. They are very slow growing and have the potential for recurrence if incompletely excised and a very remote chance for malignant changes. These can be freed from surrounding tissue without difficulty, but because of the fibrous nature of the capsule, its violation is more likely with suction technique and may result in an inadequate resection, possibly leading to recurrence. Furthermore, liposuction alone will not allow histopathological study of the swellings. Therefore, we report here the treatment of moderate (>4-10 cm) and large (>10 cm) lipomas with liposuction-assisted surgical extraction of the capsule via the same wound (1 cm in length). This capsule extraction is aimed at avoiding recurrence and evaluating the histopathological nature of these swellings. 16 patients (nine men and seven women) presented with solitary lipomas (in 11 patients) and multiple lipomas (in five patients) have been successfully treated. Methods involved 1-cm incision for both liposuction and surgical removal of the capsule. Another 1-cm counter-incision may be needed in case of large size lipomas. High patient satisfaction was achieved because of the good cosmetic results due to the small postoperative residual scar and the smooth postoperative course. There has been no recorded recurrence in six years postoperative followup.

Adult↗
PubMed2006

Pancreatic adenocarcinoma.

Adenocarcinoma is the most common malignant pancreatic tumor, affecting the head of the pancreas in 60-70% of cases. By the time of diagnosis, at least 80% of tumors are unresectable. Helical computed tomography (CT) is very effective in detecting and staging adenocarcinoma, with a sensitivity of up to 90% for detection and an accuracy of 80-90% for staging, but it has limitations in detecting small cancers. Moreover, it is not very accurate for determining nonresectability because small liver metastases, peritoneal carcinomatosis, and subtle signs of vascular infiltration may be missed. Multidetector-row CT (MDCT) has brought substantial improvements with its inherent ability to visualize vascular involvement in three dimensions. MDCT has been found to be at least equivalent to contrast-enhanced magnetic resonance imaging (MRI) for detecting adenocarcinoma. MRI can be used as a problem-solving tool in equivocal CT: MRI may help rule out pitfalls, such as inflammatory pseudotumor, focal lipomatosis, abscess, or cystic tumors. Mangafodipir-enhanced MRI reveals a very high tumor-pancreas contrast, which helps in diagnosing small cancers. Endosonography is, if available, also a very accurate tool for detecting small cancers, with a sensitivity of up to 98%. It is the technique of choice for image-guided biopsy if a histologic diagnosis is required for further therapy.

Adenocarcinoma↗
PubMed2002

The A8344G mutation in mitochondrial DNA associated with stroke-like episodes and gastrointestinal dysfunction.

We report an unusual case of encephalo-entero-myopathy associated with the A8344G mutation in the tRNA(Lys) gene of mitochondrial DNA (mtDNA). This patient had mitochondrial myopathy, multiple lipomatosis, mild hearing loss, stroke-like episodes, and paralytic ileus, but she lacked the canonical clinical features of MERRF, myoclonus, epilepsy, or ataxia. We conducted genetic, biochemical, histochemical, and immunohistochemical studies in skeletal muscle, brain, intestine, and lipoma tissue. The mutation was abundant in all tissues, and cytochrome c oxidase (COX) activity was selectively decreased in brain and small intestine. COX deficiency was also documented histochemically and immunohistochemically in the small intestine, suggesting that mitochondrial dysfunction played a role in the pathogenesis of paralytic ileus. This case illustrates an unusual and dramatic clinical phenotype of the A8344G mutation, characterized by stroke-like episodes and acute ileus.

Adult↗