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PubMed1981

Thromboxane A2 in vasotonic angina pectoris: evidence from direct measurements and inhibitor trials.

Thromboxane A2 (TxA2), an arachidonic acid metabolite causing vasoconstriction and platelet aggregation, is a putative mediator of coronary-artery vasospasm. To determine whether platelet-released TxA2 causes coronary arterial vasospasm, we measured plasma thromboxane B2 (TxB2, the inactive hydration product of TxA2) in the radial-artery and coronary-sinus blood of seven patients and performed therapeutic trials of antiplatelet agents in nine. Although coronary-sinus TxB2 levels rose from the base line approximately fivefold with spontaneous ischemia, samples drawn early in ischemia showed no rise over base-line values. Although a 150 mg dose of aspirin reduced urinary dinor-TxB2 levels by over 75 per cent, it had no effect on the course of the chronic recurrent form of angina pectoris due to vasospasm ("vasotonic angina"). Similarly, indomethacin had no effect on the frequency or duration of ischemia. TxA2 is unlikely to cause vasotonic angina, but it may be released during coronary vasospasm.

Adult↗
PubMed2000

Balloon arterioplasty of recurrent coarctation after the modified Norwood procedure in infants.

Coarctation of the reconstructed aorta after the modified Norwood procedure for hypoplastic left heart syndrome is a relatively common occurrence that can have significant adverse effects. From 1992-98, 65 infants with hypoplastic left heart syndrome or variants thereof underwent a modified Norwood procedure. Of the 50 survivors of Stage I palliation, 10 (20%) were subsequently diagnosed with clinically significant obstruction of the aortic arch at a median age of 5.1 months. Eight of these patients underwent coarctation balloon arterioplasty. All 8 patients underwent successful dilation of the coarctation, with a decrease in the pressure gradient from 43+/-20 to 4+/-5 mm Hg and an increase in aortic diameter at the site of obstruction from 3.5+/-0.5 to 7.0+/-1.0 mm (both P<0.001). There was also a significant increase in lower extremity systolic blood pressure. Although there was no immediate increase in cardiac output after dilation in most patients, ventricular function was improved in 3 patients within 24-48 hr of the procedure and in all but 2 at the most recent follow-up (median 2 years). Two patients developed bradycardia during wire placement that required cardiopulmonary resuscitation. Two others died suddenly during the follow-up period. All surviving patients have undergone subsequent palliative procedures; none have required repeat intervention for arch obstruction. We found a 20% incidence of coarctation in survivors of the modified Norwood procedure. Balloon arterioplasty is effective in relieving this obstruction, and should be carried out promptly upon diagnosis to minimize its deleterious effects on ventricular function. Balloon dilation, however, should be considered a high-risk procedure in these fragile infants, and special attention must be given to cardiovascular support.

Anastomosis, Surgical↗
PubMed1989

Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors.

Nine cases of dermatofibrosarcoma protuberans with fibrosarcomatous areas (DFSP with FS) were analyzed clinicopathologically, and the findings compared with allied tumors comprising 43 cases of ordinary DFSP, ten of superficial fibrosarcoma (SFS), and 11 of storiform variant of malignant fibrous histiocytoma (SV MFH). All but one case of DFSP with FS developed on the trunk of the body. Eight of the nine patients (89%) had one recurrence with a shorter prerecurrent interval than that of the ordinary DFSP, and a hipbone metastasis was identified in one patient. These findings reflect the unfavorable course, in contrast to the ordinary DFSP. Microscopically, the FS areas were presented either in the initial lesion (five cases) or in the recurrent one (four cases), areas showing cellularity, mitotic figures, and cytologic anaplasia that exceeded those in the DFSP areas. Dermatofibrosarcoma protuberans with FS and allied categories such as SFS and SV MFH shared various clinical and histologic features but there was a diversity in the prognosis relative to the grade of malignancy. The relationship between DFSP with FS and the allied tumors was given attention.

Adult↗
PubMed1990

DNA in chordomas of the clivus Blumenbachi.

Chordomas of the clivus are frequently denoted as malignant, mainly because of their propensity to recur, their crucial location and the fatal clinical course. Although microscopical examination commonly reveals pleomorphism, particularly of cells and nuclei, the histological assessment of malignancy is not always appropriate. The measurement of DNA could provide important information for a classification of their biological behavior (grading). Our examination of a chondroid chordoma revealed a typical diploid DNA curve within a "benign" 4C range concordant to the favorable course of this variant. Our second examination of a "typical" chordoma showed a wide pleomorphism of cell nuclei and moderate proliferation activity within a "low grade" scale. This pointed to a coming (fatal) recurrence 3.5 years after the first surgery. The third chordoma we examined presented an extraordinary 4C aneuploidy with hypertetraploid subpopulations and an increased number of bi- or poly-nucleated tumor cells with prominent nucleoli and some more mitotic figures. In 5 recurrences the DNA pattern remained principally unchanged.

Adult↗
PubMed2002

[Malignant transformation of perianal Buschke-Löwenstein tumor. Extensive abdominoperineal rectum excision and reconstruction with transpelvic myocutaneous rectus abdominis muscle flap].

INTRODUCTION: The Buschke Löwenstein tumor (giant condyloma) in its perianal variant is an extremely rare disease caused by human papilloma virus. Although of histologically benign appearance, it infiltrates and destroys the surrounding tissue. There is a high risk of local recurrence and malignant transformation. The treatment of choice is wide surgical resection. CASE: A 56-year-old woman presented with perianal giant condyloma infiltrating the rectum and vagina. The extensive soft tissue defect resulting from wide resection was filled with a transpelvic myocutaneous rectus abdominis flap. Histology showed a squamous cell carcinoma arising in the Buschke Löwenstein tumor with clear resection margins. Therefore, the patient was irradiated locally after uneventful primary wound healing. CONCLUSION: A simultaneous reconstruction of a large pelvinoperineal soft tissue defect with the transpelvic myocutaneous rectus abdominis flap allows primary healing, accelerated rehabilitation, and safe adjuvant radiotherapy without risk of serious radiation damage to the small bowel by preventing it from protruding into the pelvic defect.

Anal Canal↗
PubMed2002

[Rhabdoid meningioma. A new malignant subtype].

Of the numerous morphological variants of meningiomas only few, and among these the rhabdoid meningioma, have prognostic importance. Rhabdoid meningiomas were described for the first time in 1998 as an unusual variant with increased proliferative activity. In 2000 they have been included in the revised WHO classification of tumours of the CNS as a subtype of meningiomas with increased risk of recurrence and more aggressive growth, corresponding to WHO grade III. We report the case of a rhabdoid meningioma in a 21-year-old woman presenting as a intracerebral tumour mimicking an oligodendroglioma. The tumour showed features of a meningioma and a rhabdoid morphology with angiomatous components and was considered to be a rhabdoid meningioma. After surgery a small residual tumour remained. The patient received postoperative radiotherapy resulting in regression of the residual tumour in control examinations after 4 and 8 months. Using the presented case we discuss the differential diagnosis and prognostic significance of recognition of a rhabdoid meningioma.

Brain Neoplasms↗
PubMed1977

On the nature of syndrome delineation.

Perhaps half of all patients with multiple abnormalities have known, recognized syndromes. The other half represent unknown entities that need to be further delineated. The significance of syndrome delineation cannot be overestimated. As an unknown syndrome becomes delineated, its phenotypic spectrum, its natural history, and its risk of recurrence become known, allowing for better patient care and family counseling. The process of syndrome delineation is discussed in terms of unknown genesis syndromes of the previsionally-unique and recurrent-pattern types, and known-genesis syndromes of the pedigree, chromosomal, biochemical-defect, and environmentally-induced types. Several special syndrome categories are defined, including the chance syndrome, variant additive syndrome, association syndrome, exceptional chromosomal syndrome, and exceptional monogenic syndrome. Finally, a population definition of a syndrome is developed.

Abnormalities, Multiple↗
PubMed1995

Imaging of chondrosarcoma with histopathological and prognostic correlation. An analysis of 49 cases mainly based on plain film radiography.

PURPOSE: To investigate the course of chondrosarcoma in relation to radiographic and histopathologic findings. MATERIAL AND METHODS: 49 consecutive patients seen during an 11-year-period were analysed, including re-evaluation of their radiographic and histopathologic material. RESULTS: Forty-two patients had radiographic changes typical for cartilaginous tumours, in 37 with malignant stigmata. Seven patients had malignant changes not typical for chondrosarcoma. By histopathologic grading 16 patients had grade I, 17 grade II and 16 grade III tumours. Six of the grade II-III tumours were histopathologic variants (mesenchymal, dedifferentiated or myxoid chondrosarcomas). Surgical removal of the tumour was performed in 42 patients, 41 of whom were followed up for 0.4-11.4 years (median 3.8 years). Local recurrence occurred in 7 patients, and 11 patients developed metastases. Ten patients, 4 with local recurrence and metastases, and 6 with metastases only were dead at the end of the follow-up. The actuarial 5-year overall survival rate was 64%. The occurrence of local recurrence, metastases and death was found to be related to the histopathologic grades II and III. Atypical radiographic features only occurred in grade II-III tumours and were related to metastases and death, but not to local recurrence. CONCLUSION: Atypical radiographic findings were related to high-grade malignancy and poor prognosis.

Adolescent↗
PubMed2006

Correction of feline lipoprotein lipase deficiency with adeno-associated virus serotype 1-mediated gene transfer of the lipoprotein lipase S447X beneficial mutation.

Human lipoprotein lipase (hLPL) deficiency, for which there currently exists no adequate treatment, leads to excessive plasma triglycerides (TGs), recurrent abdominal pain, and life-threatening pancreatitis. We have shown that a single intramuscular administration of adeno-associated virus (AAV) serotype 1 vector, encoding the human LPL(S447X) variant, results in complete, long-term normalization of dyslipidemia in LPL(/) mice. As a prelude to gene therapy for human LPL deficiency, we tested the efficacy of AAV1-LPL(S447X) in LPL(/) cats, which demonstrate hypertriglyceridemia (plasma TGs, >10,000 mg/dl) and clinical symptoms similar to LPL deficiency in humans, including pancreatitis. Male LPL(/) cats were injected intramuscularly with saline or AAV1-LPL(S447X) (1 x 10(11)-1.7 x 10(12) genome copies [GC]/kg), combined with oral doses of cyclophosphamide (0-200 mg/m(2) per week) to inhibit an immune response against hLPL. Within 3-7 days after administration of >or=5 x 10(11) GC of AAV1-LPL(S447X) per kilogram, the visible plasma lipemia was completely resolved and plasma TG levels were reduced by >99% to normal levels (10-20 mg/dl); intermediate efficacy (95% reduction) was achieved with 1 x 10(11) GC/kg. Injection in two sites, greatly limiting the amount of transduced muscle, was sufficient to completely correct the dyslipidemia. By varying the dose per site, linear LPL expression was demonstrated over a wide range of local doses (4 x 10(10)-1 x 10(12) GC/site). However, efficacy was transient, because of an anti-hLPL immune response blunting LPL expression. The level and duration of efficacy were significantly improved with cyclophosphamide immunosuppression. We conclude that AAV1-mediated delivery of LPL(S447X) in muscle is an effective means to correct the hypertriglyceridemia associated with feline LPL deficiency.

Animals↗
PubMed2000

Enterocolic (lymphocytic) phlebitis: a rare cause of intestinal ischemic necrosis: a series of six patients and review of the literature.

Vasculitis of the gastrointestinal tract is known to occur as part of a systemic process but also may be present in a localized form involving only the digestive tract. We report the clinical and pathologic findings of six patients with intestinal ischemia and necrosis resulting from localized phlebitis associated with fresh and/or organized thrombosis of intramural mesenteric veins. None of the patients showed clinical or laboratory evidence of systemic vasculitis. In all cases the arteries were not involved in the inflammatory process. Follow up ranged between 2 and 15 years without recurrence necessitating reoperation. This form of intestinal phlebitis is described in the literature under different terms but lymphocytic phlebitis, granulomatous phlebitis, necrotizing phlebitis, and myointimal venous hyperplasia are probably morphologic variants of the same entity. We propose to unify the nomenclature and to use for this unusual clinicopathologic entity only the generic term of enterocolic (lymphocytic) phlebitis.

Abdomen, Acute↗
PubMed1999

Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome.

Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). The majority of the tumors occurred in infants and young children and involved the hypothalamic/chiasmatic region. The tumors were histologically similar to PA, but they were more monomorphous and more myxoid. Rosenthal fibers were not seen and only 1 of 18 tumors had eosinophilic granular bodies. At the end of the follow-up period, 6 patients were dead and 12 were alive with evidence of disease. Progression free survival (PFS) at 1 year was 38.7%. In comparison, we identified a control group of 13 classical PAs in the same age range and location as the study group. In this group, PFS at 1 year was 69.2%, which was significantly better than that for pilomyxoid tumors (p = 0.04). There was no CSF dissemination or death due to tumor progression among patients with classical PA. Eight of these patients are alive with recurrent disease, and 4 have no evidence of disease. While the monomorphous pilomyxoid tumors have some resemblance to classical PA, our results suggest that the former is a more aggressive variant or a separate entity that needs to be recognized for prognostic purposes.

Astrocytoma↗
PubMed2001

A liberatory maneuver for the treatment of horizontal canal paroxysmal positional vertigo.

OBJECTIVE: The purpose of this study was to determine the effectiveness of a new liberatory maneuver in the management of the geotropic variant of horizontal canal paroxysmal positional vertigo (HC-PPV). STUDY DESIGN: Case review. SETTING: Outpatient clinic. PATIENTS: The diagnosis of HC-PPV was based on the history of recurrent sudden crisis of vertigo associated with bursts of horizontal geotropic paroxysmal nystagmus provoked by turning the head from the supine to either lateral position. The patients were 11 men and 21 women ranging in age from 30 to 85 years (average 55.43 years), and the average duration of symptoms was 7.68 days. INTERVENTIONS: All patients were treated with a liberatory maneuver based on the hypothesis that the syndrome is caused by the presence of free-floating dense particles inside the endolymph of the posterior arm of the semicircular horizontal canal. The maneuver favors their outmigration into the utricle. Patients were reexamined immediately after the treatment and again 2 days later. MAIN OUTCOME MEASURE: The treatment outcome was considered as responsive when, after one or more liberatory maneuvers, the clinical signs of PPV disappeared at the end of physical therapy. RESULTS: The liberatory maneuver resulted in a complete remission of the positioning vertigo and nystagmus in all patients after the first session. CONCLUSIONS: This approach represents a simple and effective approach to the management of the geotropic form of HC-PPV.

Adult↗
PubMed1996

Tumor escape from immune recognition: lethal recurrent melanoma in a patient associated with downregulation of the peptide transporter protein TAP-1 and loss of expression of the immunodominant MART-1/Melan-A antigen.

In the last few years, mutiple protein target antigens for immunorecognition by T cells have been identified on human melanoma. How melanoma lesions escape from functional antigen-specific immune recognition remains poorly understood. We have identified the concomitant loss of the immunodominant T cell-defined MART-1/Melan-A antigen and downregulation of the TAP-1 gene in a recurrent metastatic melanoma that was resected in 1993. This phenotype was not observed for an earlier autologous melanoma lesion resected in 1987. The "antigen loss" could be restored in the variant tumor cell line by simultaneously providing both the MART-1/Melan-A gene (by retroviral transfer) and the TAP-1 gene (by a bioballistic approach) resulting in tumor cell sensitivity to MART-1/Melan-A-specific cytotoxic T lymphocytes. This suggests that tumor escape from immune surveillance may have occurred in vivo as a sequential result of (a) antigen loss, and (b) downregulation of the peptide-transporter protein TAP-1 expression by this patient's tumor over a 6-yr period from 1987 to 1993. These results suggest that the characterization of the T cell response to melanoma in individual patients and definition of the immunologically relevant genetic defects in tumors may be required to select the most effective therapeutic strategies for a given patient.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1999

[Current viewpoints on etiology and physiopathology of migraine].

Migraine is an inheritable disease, and the mutation causing a rare variant of migraine (familial hemiplegic migraine) has now been demonstrated. The mutated gene encodes a subunit of a brain-specific calcium channel in cell membranes. Brains of patients with recurrent migraine attacks seem to behave differently from other brains, also when examined outside attacks, in that they commonly demonstrate lack of habituation when exposed to serial stimuli. The patho-physiological process of the attack might well consist of a spreading cortical depression starting in the occipital region and gradually involving other parts of the cortex. It is primarily a cerebral process secondarily accompanied by reduced cerebral blood flow, later converting into increased flow. The pain of migraine probably is mediated by way of the trigeminal nerve which releases vasoactive peptides leading to dilatation of the greater blood vessels. During an attack there is increased metabolism in cranial parts of the brain stem, demonstrated as areas of increased blood flow in PET studies. This increased metabolism persists even when the symptoms of migraine have disappeared after drug treatment, perhaps because the migraine attacks may be generated in this region. The primary dysfunction in migraine probably is located in the brain rather than in blood vessels.

Adolescent↗
PubMed2000

[Anastomosis between the left internal spermatic and splanchnic veins. Retrospective analysis of 305 patients].

PURPOSE: To investigate the presence and the rate of anastomoses between the internal spermatic vein (ISP) and visceral veins in patients with idiopathic varicocele. MATERIAL AND METHODS: We retrospectively reviewed the venographic findings of 305 patients (age range 14-40 years; mean age 28 years) with a US diagnosis of varicocele who were submitted to sclerotherapy from 1991 to 1997. All the venographic examinations had been carried out with selective injection of the ISP for complete mapping of gonadal vessels. RESULTS: The most frequent venographic patterns (type I) was identified in 139/305 patients (45.5%), type III was found in 86/305 (28.2%), type V in 37/305 (12.1%). Type IVb was found in 32 cases (10.4%) and type IVa in 9 (2.9%). Type II was seen in 2/305 patients only (0.6%). Single or double anastomoses between the ISP and portal or systemic veins were found in 34 patients (11.1%); a single or double communication with the left colic vein was observed in 24 cases (7.8%) and a communication with the paravertebral venous system in 10 patients (3.2%). Other anastomoses with the inferior mesenteric veins and splenic veins were seen in 7 and 4 patients (2.2% and 1.3%, respectively). DISCUSSION AND CONCLUSION: The presence of vascular variants and of communications of the ISP with the visceral veins needs accurate venographic studies preliminary to sclerotherapy and a skilled interventional radiologist in order to reduce the number of recurrences.

Adolescent↗
PubMed2001

Desmoplastic melanoma.

Desmoplastic melanoma is an uncommonly encountered variant of malignant melanoma. Three histological subtypes exist: desmoplastic, neurotropic, and neural transforming. Desmoplastic melanoma commonly presents in conjunction with existing melanocytic lesions or as an amelanotic firm nodule. Local recurrences are common. Thirty patients over a 6-year period were treated at our institution for desmoplastic melanoma. All lesions were treated with local excision. Local recurrence occurred in seven patients (23%) and was treated by aggressive re-excision in each instance. Clinical regional metastasis (lymph nodal basins) were detected in two patients (6%). Distant metastasis (lung) developed in two patients (6%). Twenty-three patients (76%) were found to have desmoplastic subtype, whereas five (17%) had neurotropic subtype. Six patients (20%) had associated pigmented melanotic lesions. Average length of follow-up has been 18 months. Overall survival is 96 per cent. Presentations and histologic diagnosis can sometimes be difficult and misleading. Treatment is aggressive local excision with follow-up necessary to detect resectable recurrent lesions.

Adult↗
PubMed2000

[A clinicopathologic study on membranous basal cell adenoma].

OBJECTIVE: To investigate the clinicopathologic features and behaviour of membranous basal cell adenoma. METHODS: Twelve cases with membranous basal cell adenoma of salivary gland were analysed clinicopathologically. RESULTS: The tumors were composed of epithelial islands of various sizes and shapes. Its histologic finding was characterized by palisading of peripheral cells and excessive hyaline basal membrane. Four cases had co-existing dermal cylindromas of the scalp. In 8 cases, the tumors demonstrated multiple origin. One case showed malignant transformation and cervical lymph node metastasis. CONCLUSIONS: The differential diagnosis involved the solid variant of basal cell adenoma, basal cell adenocarcinoma, solid subtype of adenoid cystic carcinoma and basal squamous cell carcinoma. Total parotidectomy rather than superficial parotidectomy is suggested to avoid the recurrence of the tumor. A close follow up after treatment is necessary.

Adenoma↗
PubMed1992

Advanced squamous cell carcinoma of the vulva after treatment for verrucous carcinoma. A case report.

Verrucous carcinoma of the vulva, a rare variant of squamous cell carcinoma, is a locally destructive, nonmetastasizing tumor. While controversy surrounds the choice of treatment for this lesion, which frequently recurs, it is generally accepted that a recurrence is almost always local, with histologic characteristics and biologic behavior the same as those of the primary tumor. A case of squamous cell carcinoma of the vulva with involvement of the regional lymph nodes after treatment of verrucous carcinoma is reported to increase awareness of the potential biologic behavior of this uncommon tumor.

Carcinoma, Papillary↗