Intracranial primary synovial sarcoma: radiologic-pathologic correlation.

We present a very rare case of spontaneous intracranial synovial sarcoma. A 15-year-old female was admitted to our institution for a cervical spine MRI, reporting a gradual pain of the dorsal neck after an injury three months previously. After obtaining the first sequence we discovered a large skull base mass. We immediately performed a skull base MRI. MR arteriography, MR venography, CT and DSA were also performed. A large space-occupying lesion was demonstrated, extending from the right petrous bone to the occipital bone at the level of the foramen magnum and the right atlantooccipital junction. CT revealed calcifications. The MR venography demonstrated diminished flow in the right transverse and sigmoid sinus, as well in the right internal jugular vein. Otorhinolaryngological evaluation revealed a slight ipsilateral conductive hearing loss. The whole mass was macroscopically removed via a retrosigmoid, transmastoid, infratemporal approach. Postoperative course was uneventful, except for complete palsy of the ipsilateral hypoglossal nerve. The final pathological examination including immunohistochemical picture, showed findings consistent with synovial sarcoma (monophasic spindle-cell type).

Could sustained monomorphic ventricular tachycardia in the early phase of a prime acute myocardial infarction affect patient outcome?

OBJECTIVES: Sustained monomorphic ventricular tachycardia (SMVT) in the course of a prime acute myocardial infarction is not a common arrhythmia and its prognostic significance has not been specifically elucidated. The aim of the study was to estimate the prognostic implications of the occurrence of sustained monomorphic ventricular tachycardia in the early phase (<72 h) of a prime acute myocardial infarction. METHODS: We studied 690 consecutive patients admitted to the coronary care unit with a diagnosis of a prime myocardial infarction. SMVT was observed in 18 (2.6%) patients and we followed these patients for establishing the prognostic value of the arrhythmia according to the clinical characteristics. RESULTS: Patients with SMVT had a more extensive myocardial infarction based on the peak of the CK-MB isoenzyme activity (480+/-290 IU/L, vs 270+/-190 IU/L, P < .01), and higher mortality rate (40% vs 9%, P < .001). The independent predictors of SMVT were CK-MB (odds ratio [OR] 12.4), presence of complex ventricular arrhythmias (OR = 5.7), a wide QRS complex > or =130 milliseconds (OR = 4.8) and Killip class (OR = 4.8). The SMVT was itself an independent predictor of mortality (OR = 5.0). Compared with patients with ventricular fibrillation or polymorphic ventricular tachycardia, those with SMVT had a higher CK-MB activity, higher rate of wide QRS > or =130 milliseconds (33% vs 8%, P < .002), had a worse hemodynamic condition (Killip class >I:58% vs 23%, P < .04) and higher recurrence rate of ischemic events (68% vs 16%, P < .05). During the one year follow-up period, 4 patients (36.3%) of the 11 survivors from those with SMVT died of cardiac related causes. CONCLUSIONS: SMVT during the first 72 h of a prime myocardial infarction is an index of a larger healing myocardium with acute very complexed electrophysiological changes and it is an independent predictor of in-hospital mortality and a prognostic factor of a poor one year outcome.

Gliclazide treatment lowers serum ICAM-1 levels in poorly controlled type 2 diabetic patients.

OBJECTIVE: To investigate the potential effect of gliclazide on serum ICAM-1 (intercellular adhesion molecule-1) and VCAM-1 (vascular cell adhesion molecule-1) levels in poorly controlled type 2 diabetic patients. PATIENTS AND METHODS: The study included 104 patients, randomly divided into two groups. Group A comprised 53 patients (26 men) treated with gliclazide with a mean age of 67.5+/-9.9 years, a mean diabetes duration of 13.4+/-5.4 years and a mean HbA1c of 8.6+/-1.1%. Group B comprised 51 patients (25 men) treated with glibenclamide with a mean age of 66.4+/-10.9 years, a mean diabetes duration of 13.2+/-6.1 years and a mean HbA1c of 8.4+/-1.3%. A third group of 30 healthy controls (15 men) with a mean age of 63.3+/-10.4 years was also included. Serum levels of ICAM-1 and VCAM-1 were measured at the beginning of the study and after six months of treatment. RESULTS: Pretreatment serum ICAM-1 and VCAM-1 levels did not differ between groups A and B, while they were significantly higher (P=0.0001) than in healthy controls. No significant difference in HbA1c, body mass index, blood pressure control and lipid profile between the two groups was observed after the sixth month of treatment. In group A, serum ICAM-1 levels after six months of treatment were significantly reduced from 623.12+/-61.17 ng/ml to 370.14+/-49.92 ng/ml (P=0,01), while no reduction was found in VCAM-1 levels. In group B, no reduction was found in serum ICAM-1 and VCAM-1 levels after the end of the study. CONCLUSIONS: Our results suggest that gliclazide treatment reduces serum ICAM-1 levels in poorly controlled type 2 diabetic patients. This reduction is independent of the hypoglycaemic action of gliclazide.

Solitary spleen metastasis and amyloidosis in a patient with endometrial cancer.

BACKGROUND: In contrast to autopsy findings, solitary splenic metastases from solid tumors are extremely rare. It may occasionally be the first manifestation of recurrent solid cancers, and in particular of gynecologic malignancies. Secondary amyloidosis is also found in malignancy. CASE: A 52-year-woman originally diagnosed with a Stage IB, grade 2 endometrial carcinoma presented two and a half years later with a paroxysmal non-productive cough, weakness, loss of appetite and daily afternoon fever. On clinical examination wheezing on forced exhalation and painful splenomegaly was found. After an extensive work-up the patient underwent an explorative laparotomy and a splenectomy was performed. Histologic examination showed solitary spleen metastasis with amyloidosis. All symptoms resolved. As the patient had received a full course of postoperative irradiation after a total abdominal hysterectomy, six cycles of combination chemotherapy were administered. The patient remains free of recurrence, 46 months later. CONCLUSION: A case of solitary spleen metastasis with amyloidosis in a patient with endometrial cancer is presented.

A benign-looking subcutaneous metastasis from squamous cell cervical carcinoma: A case report and review of the literature.

A 30-year-old woman with a stage IIIB grade 3 squamous cell cervical carcinoma and pelvic lymph node metastases was treated with external beam radiotherapy and intracavitary brachytherapy. One month after the completion of the treatment, a rubbery, movable, and painless mass appeared in the lower third and outer aspect of her right thigh. The overlying epidermis was intact. The whole clinical picture and CT scan image indicated a benign tumor. Biopsy revealed a nonkeratinizing, grade 2-3, squamous cell carcinoma similar to the primary cervical tumor. She then received six cycles of chemotherapy (cis-platinum and 5-fluorouracil) with transient partial response. The patient died one year after the diagnosis of the disease. This is the 29th case of cutaneous metastasis from a cervical carcinoma, the seventh localized on the leg, but the first solitary one with a benign-looking appearance reported since 1855. The possible mechanism of this rare metastasis is debated and the literature is reviewed.

Radial scar of the breast.

Four cases of radial scar of the breast, primarily diagnosed as carcinoma are presented; the patients were found among 858 patients who were operated on at our Breast Unit over the last 4 years. The lesion was revealed on routine mammographic examination in 4 of our patients, while in the fourth it was found by palpation. In two of our patients mammographic examination revealed architectural distortion, in one patient micro-calcifications and in the fourth patient a stellate lesion was found. In the last two patients the lesion was localized before surgery with a hook wire. Diagnosis was established by histopathologic examination in all cases. The surgeon, the radiologist and the pathologist should be aware of this clinical entity which, in spite of its benign character, has the ability to simulate invasive carcinoma clinically, mammographically and histopathologically.

Mucoepidermoid carcinoma of the breast.

Mucoepidermoid carcinomas are very rare breast tumors. The morphology of this breast tumour is similar to its counterpart in the salivary gland. The first two cases reported were low-grade and neither patient had metastatic disease. Furthermore, none of the five patients with low-grade mucoepidermoid carcinoma reported by Fisher had axillary lymph node involvement and were alive with no evidence of disease 4 to 10 years after diagnosis and were considered of probable favourable prognosis. A high grade form of mucoepidermoid cancer might occur in the breast as it does in the salivary gland. All high-grade variant cases published had demonstrable lymph node or distant metastases and rapidly fatal outcomes. This report describes a case of high-grade mucoepidermoid carcinoma of the breast with a disease-free interval of 5 years, despite of unfavourable histologic features at the time of diagnosis.

Primary desmoid tumor of the posterior mediastinum.

We describe a unique case of a de novo desmoid tumor of the posterior mediastinum in a 21-year-old nulligravida. The tumor recurred twice despite its histologically confirmed radical extirpations. Because of the aggressive local behavior of desmoid tumors and their unusual locations, which prevent wide excision margins, we favor the early consideration of adjuvant therapy.

Endometrioid carcinoma arising in a scar of caesarean section. Case report.

Endometriosis of a surgical scar is rare and occurs mainly when a hysterectomy has been performed in the past. Development of malignancy in such a lesion is very unusual. A rare case of primary endometrioid carcinoma in the endometriosis of a caesarean scar is described.

Epithelioid angiosarcoma of the adrenal gland associated with chronic arsenical intoxication?

Epithelioid angiosarcoma is a rare tumor quite recently described. There is no accurate epidemiological study of this tumor. Among the internal organs, the liver is the one most frequently affected with angiosarcoma while there is no reference to the adrenal gland as a primary site. It is well known that the direct exposure to arsenicals (especially of vineyard cultivators) may be an important causative factor in the pathogenesis of the disease. A 59-year-old male vineyard cultivator with an epithelioid angiosarcoma of the right adrenal gland is described. The histologic characteristics as well as the immunohistochemical profile of the tumor are presented and the literature is briefly reviewed.

Hodgkin's disease in Greece: a retrospective clinicopathologic study of 78 cases.

The Rye histologic classification of Hodgkin's disease has been applied retrospectively to a series of 78 previously untreated cases of Hodgkin's disease. The patients have been followed for periods varying from 1 to 12 years. Mixed cellularity was the most common histologic type. Nodular sclerosis had an intermediate frequency, and lymphocytic predominance and lymphocytic depletion showed the lowest incidence. The sex incidence showed a male predominance in all histologic types except the nodular sclerosis, where prevalence of females was noted. The study confirms the better prognosis of the lymphocytic predominance and nodular sclerosis in comparison with the poor prognosis of the mixed cellularity and the very poor prognosis of the lymphocytic depletion. Nodular sclerosis presented with higher incidence in the mediastinum; it occurred mainly in young females and it exhibited a relatively good prognosis, findings which support the accepted view that nodular sclerosis comprises a distinct clinical and biological type of the disease. A marked difference in the survival rates in Stages I and II as compared to those of Stages III and IV was found.

Gastric duplication associated with adenomyoma.

A case of gastric duplication associated with adenomyoma including ectopic pancreatic tissue in an adult patient is reported. The histologic features, diagnostic criteria, and pathogenesis are briefly discussed.

Primary malignant mediastinal germ cell tumors: a study of three cases.

Three patients with primary malignant mediastinal germ cell tumors are presented and the literature is briefly reviewed. A great disparity exists in the literature regarding the classification and differential diagnosis of these tumors. Resection and irradiation of seminomas give relatively satisfactory results. The prognosis of malignant teratomas, embryonal cell carcinomas and choriocarcinomas is poor, regardless of the mode of treatment.