Peritoneal papillary mesothelioma in situ: BAP1 mutation with indolent behavior for 15 years.

Papillary mesothelioma in situ (PMIS) is a rare and enigmatic disease. Most instances manifest as lesions of the peritoneal serosa. The pathogenesis and behavior of peritoneal PMIS are still poorly understood, and separation from benign well differentiated peritoneal mesothelial tumors (WDPMT) may be challenging. We describe the 15-year long course of a PMIS in an adult male in which inactivating mutations of BAP1, encoding BRCA1 associated protein 1 (BAP1), were identified. Tumor samples were obtained on 2 occasions more than 8y apart. In both samples, the tumor cells were bland, with occasional focal infiltration into the stalks of larger papillary lesions. However, no invasion into subserosal adipose tissue was identified. In both samples the tumor cells did not express nuclear BAP1. Comprehensive genomic analysis of the initial tumor sample revealed a somatic inactivating mutation in BAP1 (predicted effect, Y223*) and a somatic variant of IRS2 (A701_V702insAA). An additional inactivating mutation in BAP1 (predicted effect, T69fs*5) was detected in the later sample. The patient did not receive any treatment and is still alive 15 years after initial presentation. Our experience supports the view that peritoneal PMIS may follow an indolent course for many years and prompts the question whether these tumors should uniformly be treated aggressively.

Recurrent cranial fibroblastic neoplasm in a male adolescent: case report and review of the literature.

Paediatric fibroblastic tumours are rare neoplasms, of which cranial fasciitis is the most common. We present a case of a male 7-year-old suffering from a cranial tumour preceded by a mild trauma. The tumour recurred despite radical resection within 8 months. Histologically, neither tumour could be classified as any published pathological entity. Both lesions were described as cellular fibroblastic neoplasms; in addition, the recurrent tumour featured a prominent myxoid matrix. In the 12 months following resection of the second tumour, no further disease recurrence has occurred.

Adjuvant extension of chemotherapy after neoadjuvant therapy may not improve outcome in early-stage breast cancer.

INTRODUCTION: Neoadjuvant chemotherapy (NAC) is equivalent to adjuvant therapy (AdC) in terms of survival and disease-free interval. Many institutions add AdC after NAC and surgery. However, such extended chemotherapy (ExC) is not evidence based. Study aim was to investigate if ExC improved disease-free (DFS) and overall survival (OS). PATIENTS AND METHODS: From 1998 to 2006 356 consecutive patients received NAC (45 pts), AdC (221 pts) or ExC (90 pts). We analysed these 3 groups to determine effects of ExC and to identify patients who might benefit. NAC consisted in 93% of 3-6 cycles of epirubicin+docetaxel, AdC comprised EC+/-taxanes in 72%. Median age in the NAC, AdC, and ExC-groups was 54, 56 and 52 years with follow-up of 30, 57, and 55 months. RESULTS: After NAC, 35% achieved downstaging and 10% pathologic complete remission. Surprisingly ExC seemed to result in reduction of 5-year DFS: compared to 85% and 82% after NAC and AdC, DFS was 61% after ExC (p=0.001). OS was not significantly affected (79, 91, and 78% after NAC, AdC and ExC, p=0.13). In multivariate analysis after correction for age, menopausal status, stage, grading, hormone receptors, her2-status, radiotherapy and surgery, ExC seemed to adversely affect DFS (HR 2.15, p=0.008), loco-regional and distant recurrence-rates (HR 3.0, p=0.03 and HR 2.0, p=0.02). DISCUSSION: In this single-center analysis ExC could not show advantages in terms of DFS and OS. Because multivariate analyses of retrospective data cannot account for all potential biases, these data require confirmation in randomized clinical trials. Until then, extended chemotherapy should be considered carefully. As in previous studies, no differences were found between NAC and AdC groups.

[Clinical usefulness and perspectives of sentinel node biopsy in the management of breast cancer]. / Klinische Wertigkeit und zukünftige Aspekte der "Sentinel Node Biopsy" beim Mammakarzinom.

INTRODUCTION: In view of its predictive potential, axillary nodal status plays a particularly important role in breast cancer. The concept of sentinel node biopsy (SNB) revealed an accurate method with low postoperative morbidity for staging the axilla in patients with lymph node-negative breast cancer. The aim of this study was to show that SNB alone must have a place in routine clinical work and is reliable after preoperative chemotherapy (PC) and also in patients with multicentric tumors (MC). PATIENTS AND METHOD: Between April 1997 and March 2002, a total of 300 SNBs were performed in patients with uni- or bilateral breast cancer. Of them, 45 who had completed PC and 13 with multicentric lesions underwent SNB followed by axillary dissection. The sentinel nodes (SN) were labelled with a blue dye and radioactive colloids. Median follow-up was 33.6 months (range 8.2-67.0) (StAw 1.4). RESULTS: The detection rate in the learning phase was 81.8% and later 93.5%, independently of the size of the primary tumor. Overall accuracy and negative predictive value of the first 55 patients, after PC and in those with MC came to 97.8% and 96.7%, 97.6%, 95.8% and 100%, respectively. Through focused pathologic examination, staging was improved in 11.3% of patients. A year after the procedure, one patient developed recurrence. Morbidity after SNB alone was significantly lower than after axillary dissection. CONCLUSION: Our experience shows SNB to be reliable and accurate for axillary staging in breast cancer patients. Morbidity after SNB alone was low. Conclusive data on the local recurrence rate are not yet available. Under certain conditions, SNB appears to have future clinical potential, even in patients with PC and MC, which must be confirmed in further multicentric studies. In the meantime, this method has attained definite value in the surgical therapy of breast cancer patients.

Aggressive angiomyxoma: irradiation for recurrent disease.

BACKGROUND: Aggressive angiomyxoma (AAM) is a tumor of the soft tissues predominately occurring in the genital and pelvic area with a strong propensity to local recurrences. The entity was first described in 1983. The tumor is regarded as low-grade sarcoma by some authors; its cause and pathogenesis are presently unknown. PATIENT AND METHOD: This is a case report on a 27-year-old man who underwent 4 surgical procedures of the left lower extremity because of a recurrent soft tissue neoplasm, initially (August 1993) diagnosed as a myxolipoma. The patient suffered from recurrences in February 1995, September 1996 and February 1998. The diagnosis was revised at the time of the latest recurrence. A palliative resection with macroscopic residuals left was performed in February 1998, followed by a radiation therapy with 56 Gy total dose and a concomitant administration of the radiosensitizer razoxane per os. The single radiation doses were 200 cGy 5 times a week. RESULTS: The small residuals of the tumor obviously regressed although an objective response could not be shown because the lesion was not clearly measurable. A follow-up 2 years after the radiation treatment revealed no recurrence. The time of the local control achieved as yet is already longer than any former time to regrowth between the surgical procedures. This is, to our knowledge, the first description of a therapeutic irradiation of a recurrent aggressive angiomyxoma. CONCLUSION: Radiation therapy combined with the sensitizer razoxane is able to control a recurrent AAM for an unknown time. It remains open whether a radiation treatment alone would have had a similar effect.