Israel Medical Association Journal

Background: Chondrosarcoma of the larynx is a rare tumor. The most common symptom is hoarseness. Treatment is controversial.

Objectives: To describe six patients with laryngeal chondrosarcoma from a single center.

Methods: The medical records of a major tertiary hospital were reviewed for all patients with laryngeal chondrosarcoma diagnosed and treated from 1959 to 2010. Data on background, clinical treatment and outcome were collected.

Results: Six patients, all males with a mean age of 53.3 years, were identified. Partial laryngectomy was performed in three patients, and total laryngectomy, local excision, and partial cricoidectomy in one patient each. Four patients had a permanent tracheostomy after surgery. One patient required postoperative chemotherapy and one radiotherapy. Follow-up time was 12–216 months (mean 102 months). Recurrence developed in two patients 2 and 8 years after initial treatment and was treated by salvage surgery in both patients. One patient died during the follow-up from an unrelated cause. The others are currently alive.

Conclusions: This study supports earlier reports recommending initial treatment with partial or total laryngectomy for laryngeal chondrosarcoma. Long-term follow-up for recurrence is advised. We recommend preserving the larynx, if possible, even if a permanent tracheostomy is necessary.
 

Background: Vascular insufficiency is considered to play an important role in the pathogenesis of normal-tension glaucoma (NTG). Autoregulation of blood flow in the eye has been shown to be impaired in NTG, resulting in the inability to compensate for changes in intraocular pressure or blood pressure in order to maintain adequate perfusion. Objectives: To evaluate the occurrence of combined bradycardia-hypotension during 24 hour monitoring of blood pressure and heart rate in patients with NTG.

Methods: Eleven NTG patients participated in the study. All had episodic symptoms of dizziness or lightheadedness, but were confirmed as not having a diagnosis of orthostatic hypotension. Twenty-four hour monitoring was performed with systemic blood pressure and heart rate automatically measured every 20 minutes during daytime and every hour during the night. The cardiac diastolic and systolic double products (dDP and sDP) at each reading were calculated by multiplying the heart rate by the respective blood pressure. dDP < 3600 and sDP < 5400 (corresponding to a heart rate of 60 beats/min and a blood pressure of 60 and 90 mmHg, respectively) were considered abnormally low, and dDP < 2500 and sDP < 4000 (corresponding to a heart rate 50 beats/min and a blood pressure of 50 and 80 mmHg, respectively) were considered severely abnormal.

Results: dDP was abnormally low in all 11 NTG patients on at least one occasion, the majority occurring during the night-time hours, while abnormally low sDP was present in 8 of the 11 patients. The mean cumulative duration of low dDP readings was 4.2 ± 3.2 hours. Severely low dDP readings were observed in six patients.

Conclusions: Abnormally low dDP was recorded in all NTG patients, lasting more than an hour in the majority of cases. Abnormally decreased dDP may represent a state of cardiovascular autonomic dysregulation, resulting in low ocular perfusion in certain NTG patients.

Background: Comorbid depression may play an important role in non-compliance with medical treatment among patients with chronic illnesses. Glaucoma is a potentially blinding chronic disease requiring life-long commitment to medical therapy. Patient's failure to adhere to anti-glaucoma treatment may lead to disease progression and visual loss.

Objectives: To assess the prevalence of depressive symptoms in glaucoma patients and the association between these symptoms and non-compliance with anti-glaucoma therapy.

Methods: This was a cross-sectional observational study. Compliance with pharmacotherapy was assessed with the Morisky Medication Adherence questionnaire (eight items). Screening for depression was performed by means of the CES-D scale (Center for Epidemiologic Studies Depression scale). The association between depression and compliance rates was analyzed.

Results: The study group comprised 76 glaucoma patients; 19.7% of the subjects were classified as "non-compliant" (Morisky cutoff < 10) and 21.1% suffered from depression (CES-D cutoff ≥ 16). We found a similar level of non-compliance when comparing depressed with non-depressed glaucoma patients. However, a significant correlation was observed between the level of depression and the level of non-compliance (P = 0.04).

Conclusions: Our study revealed a similar rate of depression in glaucoma patients and the general Israeli population. The presence of depression was not associated with the presence of non-compliance, yet the level of depression was associated with the level of non-compliance.

Background: Uterine sarcoma constitutes a highly malignant group of uterine tumors. It accounts for 2–6% of uterine malignancies and its incidence is 1.7 in 100,000 women. The three most common variants of uterine sarcoma are endometrial stromal sarcoma, leiomyosarcoma and carcinosarcoma. Based on relatively small case series, the literature provides little information on the risk factors, the natural course of the disease and the preferred treatment.

Objectives: To evaluate uterine sarcoma patients treated in a tertiary referral center in Israel over a 20 year period (1980–2005).

Methods: We conducted a retrospective review of the charts of 40 uterine sarcoma patients, including their tumor characteristics, stage at diagnosis, treatment modalities, follow-up and survival.

Results: The patients’ mean age was 53 years (range 32–76); 30% of the patients had carcinosarcoma, 55% had leiomyosarcoma and 15% had ESS[1]. Half of the patients presented with stage I disease, 23% stage II, 10% stage III and 15% stage IV. Thirty-nine patients were treated by surgery. Adjuvant radiotherapy was administered to 39% of the patients, adjuvant chemotherapy to 21% and combined radiotherapy and chemotherapy to 9%. The mean follow-up period was 44 months, at which time disease had recurred in 44% of the patients. The disease stage was correlated with the 5-year survival rate, which was 73.1% for stages I-II and 22.2% for stages III- IV.

Conclusions: In accordance with other larger studies our data show that the only prognostic factor that was significantly correlated with prognosis was the stage of the disease at diagnosis. Despite advances in diagnosis and treatment, survival has not improved over the last 25 years.

Background: Normal-tension glaucoma is a chronic progressive optic neuropathy of unknown etiology. Neuroimaging workup in these patients is controversial.

Objectives: To determine the value of routine neurologic and neuro-ophthalmologic evaluations in patients with NTG[1].

Methods: We conducted a retrospective review of all patients diagnosed with NTG in our institution between 2001 and 2006. Neurologic and neuro-ophthalmologic data were evaluated.

Results: Sixty-eight patients were considered suitable for the study (35 males, 33 females age range 43–90 years). Neurologic and neuro-ophthalmologic findings were normal in all of them. The computed tomography brain scan was normal in 88% and duplex carotid Doppler scan was normal in 92%.

Conclusions: Pathologic findings in neurologic and neuro-ophthalmologic assessments were uncommon in NTG. Therefore, contrary to earlier suggestions, neurologic and neuro-ophthalmologic evaluations in typical normal-tension glaucoma patients appear to have no added value.

Background: The diagnosis of gastrointestinal stromal tumors is based on documentation of c-KIT and platelet-derived growth factor-alpha receptors or specific c-KIT mutations. Before the diagnosis of GIST[1] was possible, all cases had been classified as sarcomas or benign tumors.

Objectives: To identify cases of GIST formerly diagnosed as abdominal or retroperitoneal mesenchymal tumors.

Methods: We reviewed the archive material on all surgical cases diagnosed as gastrointestinal related malignant mesenchymal tumors or GIST in our medical center during the last decade (1995–2004).

Results: Sixty-eight cases of retroperitoneal soft tissue sarcoma were identified. Thirty-eight were reconfirmed to be GIST, 19 were newly diagnosed as GIST (the hidden cases), 8 cases were re-diagnosed as mesenchymal tumors, and 3 cases of sarcoma remained sarcomas. Of all the GIST tumors, c-KIT-positive and PDGFRα[2]-positive tumors were more characteristic of primary gastric tumors, while c-KIT-positive and PDGFRα-negative tumors were found in the colorectal area. The c-KIT-negative and PDGFRα-positive cases were of gastric origin.

Conclusions: Any c-KIT-negative malignant mesenchymal mass located near the proximal gastrointestinal tract should also be stained for PDGFRα to differentiate between GIST and other soft tissue sarcomas. Practically, formerly diagnosed abdominal or retroperitoneal soft tissue sarcomas should be reviewed to identify patients with misdiagnosed GIST and thereby avoid future unnecessary and ineffective chemotherapy.

Background: Unconscious adults with spontaneous circulation after out-of-hospital cardiac arrest should be cooled to 32–34ºC (ILCOR recommendations, 2003) when the initial rhythm is ventricular fibrillation.

Objectives: To assess the technique, safety and efficacy of mild induced hypothermia in patients after OHCA[1] due to VF[2].

Methods: Patients were cooled using the MTRE CritiCoolÔ external cooling system. Cold intravenous fluids were added to achieve faster cooling in 17 patients. Data were collected prospectively and patients were analyzed according to their neurological outcome on discharge, defined by their cerebral performance category.

Results: From February 2002 to September 2006, 51 comatose VF patients with OHCA underwent MIH[3]. Treatment was discontinued early in five because of hemodynamic instability; goal temperature was reached in 98% and maintained for an average of 19.5 hours; 61% had a favorable outcome (CPC[4] 1–2) and 37% died. Improved outcome was observed with longer hypothermia time and possibly when time from collapse to return of spontaneous circulation was < 25 minutes.

Conclusions: MIH, using an external cooling system, is simple and feasible, reduces mortality and protects neurological function. Four major factors seem to influence outcome: age, co-morbidities, duration of hypothermia, and possibly the length of time from collapse to ROSC[5].

Background: The prevalence and incidence of blindness in Israel appear to be comparable to other western countries. Comparisons are difficult because of different definitions of blindness, and the uniqueness of the Israeli registry for the blind.

Objective: To characterize the population who were registered as Blind in Israel in the years 1998–2003 and estimate the prevalence and incidence of blindness by age and causes of blindness.

Methods: A retrospective review of the annual report of the National Registry for the Blind in Israel between 1998 and 2003 identified 21,585 blind persons who received a certificate for blindness. Blind persons are identified by ophthalmologists throughout Israel and referred to the Registry of the Blind if they have a visual acuity of 3/60 or worse, or a visual field loss of < 20 degrees in their better eye. This report includes prevalence data on 21,585 persons enrolled in this review still alive and living in Israel in 2003. We estimated the prevalence rate of blindness nationwide and the incidence rate for each cause of blindness for every year.

Results: The main leading causes of blindness in Israel in 1998 were (in percent of the total number of newly registered patients): age-related macular degeneration (20.1%), glaucoma (13.8%), myopic maculopathy (12%), cataract (10.4%), diabetic retinopathy and maculopathy (10.1%), and optic atrophy (7.9%), and in 2003, 28%, 11.8%, 7.4%, 6.5%, 14.4% and 6.5% respectively.

Conclusions: The results indicate that the incidence of age-related macular degeneration, diabetic retinopathy and maculopathy in Israel is increasing, while that of glaucoma, myopic maculopthy, optic atrophy and cataract is decreasing.