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[Primary malignant melanoma of the gallbladder. Case report and literature review].

An autopsy case of a 57-year-old man with primary melanoma of the gallbladder is described. The tumour appeared as a polypoid mass, measuring 4 cm in diameter. Microscopic examination revealed mostly spindle cells with vesicular nuclei and large nucleoli. Many cells stained positively for S 100 protein and HMB 45 using immunohistochemistry and they contained dark brown pigment that stained as melanin pigment with Fontana-Masson. The tumour had metastasised and affected the stomach, the duodenum, the jejunum, the lung, the brain and a bronchopulmonary lymph node. Examination of the skin and both eyes showed no abnormality. Primary malignant melanomas of the gallbladder are very rare. Only 23 cases have been described. These cases are summarized and reviewed.

Autopsy↗

Faisalabad histiocytosis mimics Rosai-Dorfman disease: brothers with lymphadenopathy, intrauterine fractures, short stature, and sensorineural deafness.

Rosai-Dorfman disease (RDD) is a rare, sporadic histiocytic disorder characterized by painless but protracted lymphadenopathy. Its etiology remains unclear. The observation of congenital disease and reports of familial cases with seven pairs of siblings including three sets of identical twins suggests a genetic predisposition in some patients with this condition. We now report two brothers of consanguineous Palestinian parents, whose lymphadenopathy, lymph node histology, and polyclonal hypergammaglobulinemia indicated RDD. The presence of intrauterine fractures, short stature, and sensorineural hearing impairment suggested a rare familial form of the disorder. Moynihan et al. recently described a Pakistani family with a familial histiocytic disorder highly reminiscent of the brothers reported here, whose lymph node morphology was apparently consistent with RDD as well. The presence of sensorineural deafness, short stature, and joint contractures, however, suggested a separate, rare autosomal recessive syndrome referred to as Faisalabad histiocytosis, after the family's place of origin. We believe that the brothers described here represent a second family with Faisalabad histiocytosis, which mimics RDD histologically.

Abnormalities, Multiple↗

Combined physical therapy for lymphedema evaluated by fluorescence microlymphography and lymph capillary pressure measurements.

The treatment of patients with lymphedema is still controversial. Combined physical therapy with manual lymph drainage and compression therapy is most frequently used to reduce lymphatic leg swelling. However, objective evidence is rare that this empirical form of treatment has a scientific basis. In a prospective study fluorescence microlymphography and pressure measurements in cutaneous lymph capillaries were used to assess objectively the effect of combined decongestive physical therapy on abnormal microlymphatic dynamics in lymphedema. 12 patients with primary and secondary lymphedema were studied before treatment, after 2 weeks of intensive physical therapy and 3 months of continuing compression and ergotherapy. After 2 weeks of intensive manual lymph drainage and compression bandaging (phase 1) microlymphatic hypertension (12.8 +/- 5.7 mm Hg) was significantly (p = 0.01) reduced to a mean lymph capillary pressure of 5.9 +/- 4.5 mm Hg. More than 3 months later after continuing compression lymph capillary pressure (3.2 +/- 5.2 mm Hg) was still significantly (p = 0.03) reduced. Simultaneously the maximum spread of the fluorescent contrast medium in the superficial lymph capillary network decreased significantly (p = 0.01) from 21.3 +/- 14.3 to 11.3 +/- 4.8 mm. Accordingly the clinical condition improved, and the mean circumferences of the forefoot and ankle were significantly (p < 0.05) reduced. Combined decongestive physical therapy is an effective treatment for lymphedema which results in a normalization of microlymphatic hypertension and an improvement of the clinical appearance.

Adult↗

The role of (111)In Capromab Pendetide (Prosta-ScintR) immunoscintigraphy in the management of prostate cancer.

(111)In Capromab Pendetide (ProstaScintR) is a whole murine antibody that is reactive with prostate specific membrane antigen (PSMA), a glycoprotein on the surface of normal and abnormal prostate epithelium. It has proven to be of great value in assisting management decisions in prostate cancer patients who initially present with high risk for metastatic spread, or who develop a picture of recurrent disease after surgery or radiation therapy. Patterns of metastatic lymphatic spread have correlated well with autopsy reports in the literature. Unfortunately, other imaging study and/or histologic confirmation of scintigraphic findings has been difficult to obtain. Prostascint's role in predicting durable complete response (DCR) in postoperative patients having salvage radiotherapy to their prostate fossa is very promising. Further investigative work in larger patient populations is needed to confirm these early results.

Antibodies, Monoclonal↗

Clinical significance of isolated scintigraphic sternal lesions in patients with breast cancer.

Isolated scintigraphic sternal lesions are rare. In a retrospective review of the bone scan results of 1,104 patients with breast cancer, 34 individuals (3.1%) presented with this abnormality. Of these foci, 26 (76%) were found to represent metastatic disease. There did not appear to be a relationship with axillary lymph node metastasis, the quadrant involved by primary breast tumor, or selected serum chemistries. These sternal lesions may be associated with regional lymphatic tumor spread rather than hematogenous seeding and therefore could be considered a local recurrence.

Bone Neoplasms↗

Response to treatment in acute non-lymphatic leukaemia: prognostic value of colony forming and colony stimulating capacities of bone marrow and blood cells compared to other parameters.

Growth of bone marrow and mononuclear white blood cells (MWBC) in soft-agar cultures was studied in 26 patients with untreated acute non-lymphocytic leukaemia (ANLL). Marrow and MWBC from 30 healthy volunteers served as controls. All ANLL patients revealed an abnormal growth in vitro. Patients with an increased number of clones in marrow cultures and large cluster predominance ('excessive growth') responded poorly to therapy with only one of 10 patients entering remission. On the contrary, only two of the 15 patients with a decreased clone number ('low growth') failed to achieve remission. The number of colonies and clusters in both bone marrow and blood cultures was significantly lower at presentation in patients who later entered remission than in those who did not. The correlations between the number of colonies and clusters in the blood and the marrow cultures were statistically significant. No significant correlations were found between prognosis or colony formation, on one hand, and the production of colony stimulating activity (CS)bA), by bone marrow and blood cells of ANLL patients, on the other. Nor could such correlations be found between prognosis, blood cell counts, and age. It is concluded that the growth pattero cytostatic drugs and particularly in selecting patients with a high probability to respond poorly to current cytostatic regimes.

Acute Disease↗

[A case report of distant lymph nodes metastases from prostate cancer imaged with 201Tl and 99mTc-MIBI].

Prostate cancer most often metastases to regional lymph nodes and bones by hematogeneous or lymphatic spread. The authors present a rare case of metastatic prostate cancer to supradiaphragmatic lymph nodes that were detected on 201Tl and 99mTc-MIBI imaging and confirmed on a CT scan. An 81-yr-old man with bilateral painful cervical lymphadenopathies was referred to our hospital with suspected thyroid cancer. The US and thyroid scan indicated no abnormalities in his thyroid gland. Both 201Tl and 99mTc-MIBI scans showed multiple areas of abnormally increased radioactivity in both supraclavicular, anterior mediastinum, and bilateral hilar regions. A CT scan also revealed multiple lymphadenopatheis in the same regions as radionuclide scans. Prostate cancer was diagnosed from the results of immunohistochemical staining for PSA examination of a biopsy specimen of the mediastinal lymph node. The serum PSA concentration was markedly elevated at 490 ng/ml (normal, < 40 ng/ml). Both 99mTc-HMDP bone and 67Ga scans were normal. All supradiaphragmatic lymph nodes on CT images disappeared 2 months after subcapsular orchiectomy and endocrine treatment with Bicalutamide. Metastatic prostate cancer should be considered when metastatic adenocarcinoma is discovered in the supraclavicular lymph nodes of elder men.

Adenocarcinoma↗

The spleen in the spleen syndrome: the association of splenoma with hematopoietic and neoplastic disease--compendium of cases since 1864.

Splenomas have been described as benign congenital growths. We report six cases of splenomas, all in males. Three had hairy cell leukemia, one had Hodgkin's disease, and one had myeloid metaplasia. Average splenic weight was 1629 gm, and all had multiple splenomas (average diameter 1.89 cm). Splenomas bulged above the cut splenic surface, were pseudoencapsulated, and showed involvement of the underlying disease. A re-evaluation of 105 prior cases are adult (86%; age: 49), with 56% associated with neoplastic disease or hematologic abnormalities. Average spleen weight was 626 gm, and 22% contained multiple splenomas. We conclude that splenomas are most likely an acquired proliferative process strongly associated with neoplastic disease or hematologic abnormalities. Splenomas can also be congenital growths (hamartoma; 26% of the series), which are of little importance.

Adult↗

Mesenteric lymphadenopathy in children examined by US for chronic and/or recurrent abdominal pain.

BACKGROUND: Children with recurrent abdominal pain often undergo US to confirm or exclude organic disease. OBJECTIVE: To assess the prevalence of mesenteric lymphadenopathy on US in these children. MATERIALS AND METHODS: We prospectively studied 189 children with recurrent abdominal pain with US of the abdomen, using graded compression. The results were compared with 73 children in a control group. The children in both groups were divided into three age groups. The size, number, morphology and location of mesenteric lymph nodes were noted, as well as additional findings. Pediatricians followed the patients from 3 months to 1 year, and a repeat US study was done in 30 children. RESULTS: Mesenteric lymphadenopathy was present in 116 of 189 children (61.4%), with the greatest prevalence in boys in the younger age groups. The location of the nodes was mainly in the right lower quadrant. In the control group, 7 of 73 children had mesenteric lymphadenopathy, a significantly lower prevalence than in the study group ( P<0.001). Additional findings, apart from lymphadenopathy, were present in 27 (14.2%) of the 189 children in the study group, and in 5 (6.8%) of the 73 children in the control group. CONCLUSION: Mesenteric lymphadenopathy is a common, and often the only abnormal, finding on US in children with recurrent abdominal pain.

Abdominal Pain↗

Change of sonographic findings on cervical lymph nodes before and after preoperative radiotherapy.

The aim of this study was to assess the changes in the power Doppler sonographic findings in patients with oral cancer undergoing chemotherapy and radiotherapy. We performed US examinations on 187 cervical lymph nodes (71 metastatic and 116 reactive nodes) excised from 52 patients before and after preoperative therapy. On Power Doppler images, we calculated the vascular index (VI) and evaluated the vascular pattern. We also assessed the diagnostic power using receiver operating characteristic (ROC) curve analysis. Irradiation caused an increase of the VI and better visualization of the vessels within the lymph node in the reactive nodes; however, in the metastatic nodes, the VI was not significantly different between that before and after irradiation. When the reader observed the images before irradiation, the area under an ROC curve (Az values) observed by B-mode sonography were closely similar to those obtained by B-mode plus power Doppler sonography. With both images before and after irradiation, the Az value obtained by B-mode plus power Doppler sonography was higher than that by B-mode sonography alone. After irradiation, the enhanced Doppler signals contributed to a better visualization of the vessels and a better detection of any vascular abnormalities.

Adult↗

[Nasolacrimal ducts and the dry eye].

BACKGROUND: The knowledge regarding the pathogenesis, diagnosis, and treatment of dry eye has made large advances in recent years. However, many questions are still unanswered. Although the nasolacrimal ducts are part of the tear system they have been paid nearly no attention regarding dry eye. METHODS: The present knowledge about the nasolacrimal ducts is presented and discussed in a context with dry eye. A PubMED search was conducted for articles published from 1966 to the present; in addition, review articles as well as book chapters were considered and discussions with investigators in the field were performed. RESULTS: As a draining and secretory system, the nasolacrimal ducts play a role in tear transport and non-specific immune defense. Moreover, components of tear fluid are absorbed in the nasolacrimal passage and are transported into a vascular system that surrounds the nasolacrimal sac and nasolacrimal duct. This system is comparable to a cavernous body and it is connected to the blood vessels of the outer eye. Also it is subject to vegetative control. Organized mucosa-associated lymphoid tissue (MALT) is present in the nasolacrimal ducts displaying the cytomorphological and immunophenotypic features of MALT. CONCLUSIONS: The normally constant absorption of tear fluid components into the blood vessels of the surrounding cavernous body that are connected to the blood vessels of the outer eye, could be a feedback signal for tear fluid production, which comes to a halt if these tear components are not absorbed. Thus, dry eye could be initiated. Defective stimulation of tear duct-associated lymphoid tissue (TALT) could result in abnormal immune deviation at the ocular surface leading to an autoimmunological response that causes dry eye pathology.

Autoimmune Diseases↗

Cardiac and coronary arterial involvement in infants and children from New England with mucocutaneous lymph node syndrome (Kawasaki disease). Angiocardiographic-echocardiographic correlations.

Forty-four patients, aged 6 months to 11 years, meeting the clinical criteria for mucocutaneous lymph node syndrome (Kawasaki disease) were studied from March 1980 to March 1981. A protocol was designed for evaluation of cardiac involvement including electrocardiogram, chest X-ray film, cardiac enzyme determinations and M mode and two dimensional echocardiograms. Aspirin was given during the acute febrile period as an anti-inflammatory agent in a dosage of 100 mg/kg per day; followed by 30 mg/kg per day for 10 weeks or longer if platelet counts were elevated. Angiocardiography was performed in 38 patients. Twenty-one patients (48 percent) showed abnormal M mode echocardiographic findings (flat or greatly decreased ventricular septal motion, pericardial effusion or decreased left ventricular function) during the acute febrile stage. Coronary artery disease developed in seven patients, all of whom had abnormal M mode echocardiographic abnormalities in the acute stage. Five patients had coronary aneurysms and two patients had dilated coronary arteries. Two dimensional echocardiograms identified coronary arterial lesions accurately if present proximally but failed to detect aneurysms beyond 1.5 cm from the aortic root. The study shows that serial M mode and two dimensional echocardiograms provide sufficient information to rule out cardiac involvement in Kawasaki disease. Echocardiograms should be obtained every 3 to 4 days during the acute febrile stage because all patients with coronary artery disease detected with angiography had abnormal echocardiographic findings during that period.

Angiocardiography↗

Cyclophosphamide-induced changes in the MRL-lpr/lpr mouse: effects upon cellular composition, immune function, and disease.

MRL-lpr/lpr mice develop massive lymphadenopathy with excessive proliferation of T cells and associated immune abnormalities. To examine whether to not the disease process is intrinsic and irreversible, an immunomodulatory drug, cyclophosphamide, was administered to 16-week-old, sick MRL-lpr/lpr mice. Analysis of lymph node cells by flow cytometry from injected and control MRL-lpr/lpr mice indicated that cyclophosphamide had a marked effect upon the lymphoid cellular composition. Whereas control lymph nodes had a very large number of abnormal dull Ly 1+ T cells and very few other cells, the cyclophosphamide-injected mice had normal T and B cells. The immune responses to a T-cell-dependent antigen, sheep red blood cells, and a T-cell mitogen, concanavalin A, were normalized in cyclophosphamide-injected mice as compared to controls. In addition, injected mice had prolonged survival, decreased arthritis, markedly reduced adenopathy and splenomegaly, improved renal histology, and significantly diminished autoantibody levels. This study suggests that the disease and associated immune abnormalities of MRL-lpr/lpr mice are reversible by selective elimination of the abnormal dull Ly 1+ T cells.

Animals↗

Effects of lithium carbonate on rat seminiferous tubules: an ultrastructural study.

Lithium salts are commonly used for treatment of bipolar disorder but prolonged treatment with therapeutic doses induces substantial toxic effects. In the present study we examined the effects of lithium carbonate on the ultrastructure of rat seminiferous tubules. Rats were exposed to lithium carbonate at doses of 35 mg/kg/day for 21 days. After lithium treatment, the tunica propria widened and folded together with convolutions of the basement membrane, myoid cells and lymphatic endothelium. In the seminiferous epithelium loss of germ cell attachment and appearance of expanded intercellular spaces between spermatogenic cells were observed. Early stages of spermatogenic cells showed nuclear protrusions or swellings because of an extensive enlargement of the outer nuclear membrane. Round spermatids exhibited abnormally shaped acrosomes and dilation of the subacrosomal space. Many abnormal, degenerated late spermatids with random orientation were seen towards the basal and adluminal compartments of the seminiferous epithelium. In addition, spermatids exhibited alteration in F-actin bundle ectoplasmic specialization and contained many mitochondria-associated granular bodies.

Animals↗

DNA-ploidy studies in a keratocyst undergoing subsequent malignant transformation.

Using techniques that allow flow cytometry to be performed on tissue stored in paraffin wax, we have examined the DNA content of cells from an odontogenic keratocyst which underwent malignant transformation. On histological examination, the primary lesion was an odontogenic keratocyst showing dysplastic changes within the squamous epithelium. Flow cytometry revealed a prominent, abnormal DNA stemline, which was also present in the subsequent squamous cell carcinoma.

Carcinoma, Squamous Cell↗

Villous atrophy with crypt hyperplasia in malignant histiocytosis of the nose.

Intestinal changes, mainly in the jejunum, were investigated in 13 cases of malignant histiocytosis at necropsy and who had presented as lethal midline granuloma. Villous atrophy with crypt hyperplasia was observed in all cases, and a proliferation of atypical histiocytes was observed in seven cases. In the remaining cases, histiocytes with normal morphology increased in number. These findings showed that a prolonged abnormal proliferation of histiocytes was present in the small intestine of these cases concurrently with the nasal lesions.

Adult↗

[Splenectomy in immune thrombocytopenia and other hematological diseases].

The Authors report a retrospective study of 74 splenectomies performed for hematologic diseases. The role of splenectomy has changed over recent years with increased indications for immune thrombocytopenic purpura (ITP). The aim of this study was to assess indications to surgery in relation to clinical presentation with typical hemorrhagic features or severe thrombocytopenia only; interval between onset of symptoms and splenectomy; failure of medical management and complications from steroid administration; scintigraphic studies predictive of response to splenectomy and preoperative treatment in patients with severe thrombocytopenia were also studied. The Authors reported response rates to splenectomy of 84% without mortality and only 11% of postoperative complications. These results encouraged to surgery for treatment of those patients with severe thrombocytopenia, who fail to obtain remission or develop serious complications after medical therapy. The splenectomy cured severe thrombocytopenia also in some patients with acquired immunodeficiency (HIV+). Moreover the Authors discuss the indications in patients with chronic lymphatic leukaemia and lymphoma diseases. In selected patients the splenectomy has the potential to relieve symptoms due to splenomegaly, correct cytopenias, specify hystological diagnosis and modify the disease course of malignant lymphomas. In fact splenomegaly sometimes complicated the course of malignant lymphomas because of hematologic abnormalities that are inconsistent with active chemotherapy.

Acquired Immunodeficiency Syndrome↗