Justine S. Broecker

Unusual Presentation of Rosai-Dorfman Disease (RDD) in the Bone in Adolescents

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare idiopathic histiocytic disorder. The usual presentation of RDD is painless bilateral cervical lympthadenopathy. Extranodal RDD with lymphadenopathy occurs in almost 50% of patients but extranodal RDD, without lymphadenopathy, is very rare. Isolated RDD in the bone occurs in only 2% of patients but it is histologically similar to its nodal counterpart. There are only 14 previously reported cases of RDD in the bone without lymph node involvement in children. Here we describe two new patients—one with rib and lung involvement and the other with multi-osseous involvement.

Nephrogenic adenoma of the bladder in a prune belly syndrome patient: case report and review of the literature.

Nephrogenic adenoma (NA) is a rare lesion of the urinary tract widely considered to be a metaplastic response to urothelial injury. Herein, we present the case of an 8-year-old male with prune belly syndrome who presented with gross hematuria. Investigation revealed a bladder mass; however, upon cystoscopic examination, multiple polypoid lesions were identified. Microscopic examination revealed NA of the bladder. To our knowledge, this is the second reported case of NA of the bladder in association with prune belly syndrome.

The Expanded Use of Autoaugmentation Techniques in Oncoplastic Breast Surgery

Background: Autoaugmentation techniques have been applied to oncoplastic reductions to assist with filling larger, more remote defects, and to women with smaller breasts. The purpose of this report is to describe the use of autoaugmentation techniques in oncoplastic reduction and compare the results with those of traditional oncoplastic reduction. Methods: The authors queried a prospectively maintained database of all women who underwent partial mastectomy and oncoplastic reduction between 1994 and October of 2015. The autoaugmentation techniques were defined as (1) extended primary nipple autoaugmentation pedicle, and (2) primary nipple pedicle and secondary autoaugmentation pedicle. Comparisons were made to a control oncoplastic group. Results: There were a total of 333 patients, 222 patients (67.7 percent) without autoaugmentation and 111 patients (33 percent) with autoaugmentation (51 patients with an extended autoaugmentation pedicle, and 60 patients with a secondary autoaugmentation pedicle). Biopsy weight was smallest in the extended pedicle group (136 g) and largest in the regular oncoplastic group (235 g; p = 0.017). Superomedial was the most common extended pedicle, and lateral was the most common location. Inferolateral was the most common secondary pedicle for lateral and upper outer defects. There were no significant differences in the overall complication rate: 15.5 percent in the regular oncoplastic group, 19.6 percent in the extended pedicle group, and 20 percent in the secondary pedicle group. Conclusions: Autoaugmentation techniques have evolved to manage complex defects not amenable to standard oncoplastic reduction methods. They are often required for lateral defects, especially in smaller breasts. Autoaugmentation can be performed safely without an increased risk of complications, broadening the indications for breast conservation therapy. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

An atypical presentation of Paget’s Disease of the breast without nipple involvement: Case report and review of the literature

We present a case of a 63 year-old Caucasian female who developed a right breast skin lesion discrete from the nipple that was subsequently diagnosed as Pagets Disease of the breast (PDB). Imaging did not reveal an underlying breast cancer or involvement of the nipple. The patient underwent a segmental mastectomy preserving the nipple and final pathology demonstrated residual Pagets disease of the skin and did not reveal any additional underlying breast carcinoma. To our knowledge, this case represents the first reported diagnosed case of isolated PDB without nipple involvement.

Primary Pediatric Breast Lesions: Comparing the Prevalence of Malignancies in the Middle East with North America; Review of 1031 Cases

ABSTRACT Benign breast masses are uncommon but are becoming more recognized among the pediatric population. Malignant breast lesions are very rare. The aim of our study was to review and compare the demographics, clinical presentation, treatment, and outcomes of breast lesions, including primary malignancies, and to discuss theories that may explain why there is an increased rate of breast cancers diagnosed at a younger age in the Egyptian population. A total of 1031 cases were reviewed. Comparisons were made between the Egyptian (n = 846) and US (n = 185) cohorts. There were 30 (3.5%) malignant tumors in the Egyptian group with 17 (2%) deaths versus 3 (1.6%) malignant tumors in the US group with 2 (1%) deaths (p = 0.247). The relative risk of breast cancer in the Egyptian group was 2.16 (95% confidence interval (CI): 0.67–7.01) compared with the US group. The trend for increased risk of breast cancer in Egypt may be due to delayed diagnosis because of decreased awareness.

Neoadjuvant Therapy Combined With Oncoplastic Reduction for High-Stage Breast Cancer Patients

Objective Oncoplastic surgery has been shown to be a good alternative to breast conservation surgery (BCS) alone for patients with breast cancer. Its role in patients with advanced disease is unclear. In this study, we evaluate the safety of oncoplastic BCS (OBCS) in patients who received neoadjuvant therapy (NT) for high stage breast cancer. Methods The oncologic outcomes of consecutive patients classified as high stage (>T2 or at least N1) who received NT followed by BCS at EUH by a single breast surgeon (T.M.S.) from September 2004 until June 2015 were compared with those who received BCS combined with an oncoplastic reduction. Patients were surveyed using the BREAST-Q to determine their satisfaction after surgery. Results A total of 87 patients were included in this series. The mean initial tumor size (4.37 vs 2.56 cm), the weight of the surgical specimen, and the post-NT tumor size were all larger in the OBCS group as compared with BCS alone (1.54 vs 1.29 cm). The mean follow-up was 44 months. The average percent reduction in tumor size in response to NT was slightly greater in the OBCS group (61 vs 52%). Oncologic outcomes were similar for OBCS reduction and BCS groups, respectively: positive margin rate, reexcision rate, completion mastectomy rate, local recurrence rate, and 5-year DSS. Patient satisfaction was similar between the 2 groups. Conclusions The oncoplastic approach in high stage patients treated with neoadjuvant systemic Powered by Editorial Manager and ProduXion Manager from Aries Systems Corporation therapy appears to be as safe and effective when compared to BCS alone. Oncoplastic BCS paired with NT broadens the indication for BCS for patients with larger tumor size.

Paracrine Effects of Leydig Cell Nodular Hyperplasia, Two Case Reports: A Neglected Phenomenon

ABSTRACT Leydig cell nodular hyperplasia (LCNH) is a lesion that is less characterized than the familiar Leydig cell tumors. The paracrine effects of these lesions on adjacent gonadal stroma have not been widely documented. We present two cases of precocious puberty in pre-pubertal boys found to have a single LCNH with adjacent focal maturation of the seminiferous tubules. Blood tests showed elevated serum testosterone and dehydroepiandrosterone (DHEAS). Ultrasound revealed unilateral testicular enlargement with irregular echogenicity. Radical orchiectomy was performed. Histologically Leydig cell nodular proliferation without destruction of surrounding tubules was seen. Mature seminiferous tubules undergoing spermatogenesis were noted adjacent to the lesion, while away from the lesion seminiferous tubules were as expected in pre-pubescent boys. These cases emphasize the potential presence of both paracrine and endocrine effects in Leydig cell nodular hyperplasia. However, instances of the endocrine effects of hyperplastic Leydig cell lesions are more widely reported than the paracrine effects.