Israel Medical Association Journal

Background: Granulomatous lobular mastitis (GLM) is a rare disorder that can clinically mimic breast carcinoma. The recommendation for diagnosis and treatment of GLM has not yet been established. 

Objectives: To assess a series of GLM patients, including their clinical presentation, diagnosis, treatment and outcome. 

Methods: We retrospectively analyzed the clinical data and treatment of 17 female patients with biopsy-proven GLM. Breast tissue was obtained by a core needle biopsy (15 patients) or open biopsy (2 patients). Images were reviewed by an experienced radiologist.

Results: The mean age of the patients at diagnosis was 44.6 ± 12.6 years. Five patients (29%) presented with bilateral disease, and seven (41%) presented with a mass, suggesting the initial diagnosis of breast carcinoma. Treatment comprised observation alone (23%), antibiotics (58.8%) and/or corticosteroids (with or without methotrexate) (35%). At the end of the study 70.6% of the patients demonstrated complete remission. None of the patients developed any systemic (granulomatous) disease or breast carcinoma during the follow-up period (4.7 ± 3.8 years). 

Conclusions: Core needle biopsy is mandatory for the diagnosis of GLM and the exclusion of breast carcinoma. The recommended treatment modalities are observation alone or corticosteroids; surgery should be avoided. GLM is a benign disease with a high rate of resolution and complete remission.

 

Abstract

Background: Only a few studies have compared the accuracy of magnetic resonance enterography (MRE) and computed tomography enterography (CTE) in the diagnosis of Crohn's disease and its complications.

Objectives: To compare the sensitivity of MRE and CTE analysis in their ability to detect, sign-by-sign, 10 classical imaging signs of Crohn's disease.

Methods: The study group comprised 42 biopsy-proven Crohn's disease patients who underwent both CTE and MRE within an average period of 6 weeks. Agreement between the two modalities in detecting the 10 most significant radiological signs of CD was evaluated using the Kappa index. The sensitivity of MRE and CTE was calculated using a standard of reference composed of all the findings seen by CTE and/or MRE. We analyzed MRE and CTE sensitivity separately in two groups, according to the time interval between the examinations.

Results: Agreement between CTE and MRE was more than 70% in 8 of the 10 signs: mural thickening, phlegmon, stenosis, skip lesions, mucosal stratification, fistula, abscess, and creeping fat. The Kappa level of agreement values for CTE versus MRE varied between substantial for phlegmon and skip lesions; moderate for fistula, creeping fat, abscess and mural thickening; and fair for stenosis and dilatation. CTE detected more findings than MRE, except for creeping fat and fistula. There was no significant difference in the sensitivity of CTE and MRE in the two groups defined by the time interval (time < 1.5 and time > 1.5 months) except for detection of dilatation.

Conclusions: Almost all imaging signs of Crohn's disease were detected equally well by both modalities regardless of the time interval between examinations. We therefore consider MRE to be reliable for imaging and follow-up in patients with Crohn's disease who may need recurrent imaging.

Fungal diseases affect a large proportion of the population, ranging in severity from mild superficial infections to life-threatening invasive diseases. Estimates of disease burden are needed to inform public health policies. We estimated the incidence of fungal diseases in Israel based on published surveillance data and risk-based calculations using previously established models. Deaths associated with fungal diseases were estimated from local surveillance data (candidemia) and published reports (invasive aspergillosis). Candidemia was estimated in 649 persons/year and invasive aspergillosis in 254 persons/year; the associated mortality was 2.75 and 0.96 per 100,000 population/year, respectively. Recurrent Candida vulvovaginitis occurs in 130,440 women annually. National incidence rates of cryptococcosis, pneumocystis pneumonia and mucormycosis could not be reliably estimated. Single-center data-derived estimates yielded 24, 26 and 20 cases/year, respectively. Allergic bronchopulmonary aspergillosis, asthma with fungal sensitization and allergic fungal sinusitis affect 8297 (range 2323–11,615), 14,372 (14,372–17,965) and 39,922 (15,969–183,643) persons, respectively. In Israel, candidemia and invasive aspergillosis rank high among infection-related causes of mortality. Allergic fungal diseases cause chronic or recurrent symptoms in a large population and may contribute to asthma-related hospitalization and death. These general estimates should serve as a primer for future efforts to study fungal epidemiology. 

 

Abstract

Background: Scleroderma lung disease (ILD-SSc) is treated mainly with cyclophosphamide (CYC). The effectiveness of CYC was judged after 12–24 months in most reports.

Objectives: To analyze the effect of monthly intravenous CYC on pulmonary function tests including forced vital capacity (FVC) and diffusing lung capacity (DLCO), as well as Rodnan skin score (mRSS), during long-term follow-up.

Methods: We retrospectively collected the data on 26 ILD-SSc patients who began CYC treatments before 2007. Changes in FVC, DLCO and mRSS before treatment, and at 1, 4 and 7 years after completion of at least six monthly intravenous CYC treatments for ILD-SSc were analyzed.

Results: Mean cumulative CYC dose was 8.91 ± 3.25 G. More than 30% reduction in FVC (0%, 8%, and 31% of patients), DLCO (15%, 23%, 31%), and mRSS (31%, 54%, 62%) at years 1, 4 and 7 was registered. During the years 0–4 and 4–7, annual changes in FVC, DLCO and mRSS were 3.2 vs. 0.42% (P < 0.040), 4.6 vs. 0.89% (P < 0.001), and 1.8 vs. 0.2 (P = 0.002). The greatest annual FVC and DLCO reduction over the first 4 years correlated with mortality (P = 0.022). There were no differences in the main variables regarding doses of CYC (< 6 G and > 6 G).

Conclusions: In patients with ILD-SSc, CYC stabilized the reduction of FVC during treatment, but this effect was not persistent. The vascular characteristic of ILD-SSc (DLCO) was not affected by CYC treatment. CYC rapidly improved the mRSS. This effect could be achieved with at least 6 G of CYC. Higher rates of annual reduction in FVC and DLCO in the first 4 years indicate the narrow window of opportunity and raise the question regarding ongoing immunosuppression following CYC infusions.

 

Abstract

Background: Renal hemangiomas are rare benign tumors seldom distinguished from malignant tumors preoperatively.

Objectives: To describe the Memorial Sloan-Kettering Cancer Center (MSKCC) experience with diagnosing and treating renal hemangiomas, and to explore possible clinical and radiologic features that can aid in diagnosing renal hemangiomas preoperatively.

Methods: Patients with renal hemangiomas treated at MSKCC were identified in our prospectively collected renal tumor database. Descriptive statistics were used to describe the patient characteristics and the tumor characteristics. All available preoperative imaging studies were reviewed to assess common findings and explore possible characteristics distinguishing benign hemangiomas from malignant renal tumors preoperatively.

Results: Of 6341 patients in our database 15 were identified. Eleven (73%) were males, median age at diagnosis was 53.3 years, and the affected side was evenly distributed. All but two patients were treated surgically. The mean decrease in estimated glomerular filtration rate (eGFR) after surgery was 36.3%; one patient had an abnormal presurgical eGFR and only two patients had a normal eGFR after surgery. We could not identify radiographic features that would make preoperative diagnosis certain, but we did identify features characteristic of hepatic hemangiomas that were also present in some of the renal hemangiomas.

Conclusions: Most renal hemangiomas cannot be distinguished from other common renal cortical tumors preoperatively. In select cases a renal biopsy can identify this benign lesion and the deleterious effects of extirpative surgery can be avoided.