Retroperitoneal sarcomas: from diagnosis to treatment. Case series and review of the literature.

BACKGROUND: Retroperitoneal sarcoma is a rare malignancy arising from mesenchymal cells, most commonly presented as an abdominal mass and is associated with poor prognosis. The most effective treatment modality for retroperitoneal sarcomas is complete surgical resection, including sometimes adjacent organs infiltrated by the tumor. Radiotherapy is frequently applied and has shown some benefit, while the role of chemotherapy and molecular-targeted agents is still not clear. Local recurrence is common for retroperitoneal sarcomas and still remains the main cause of death. The major factors associated with the overall survival are tumor grade, histological subtype, complete macroscopic excision and multifocality. AIM: To report our experience via the presentation of patients with retroperitoneal sarcomas managed in our department during the period 2014-18; and to review the current literature. PATIENTS AND METHODS: Eight patients appeared with chronic non-specific complaints including abdominal distension and changes in bowel or bladder habit, while one patient presented with acute abdominal pain due to mass rupture. All of the patients underwent surgical resection of the tumor. RESULTS: Among the patients, seven were operated for primary disease and one only for recurrent. The most common histologic type was liposarcoma (well-differentiated, dedifferentiated), found in five patients; followed by leiomyosarcoma found in two cases. Fibrous histiocytoma was found in only one case. The masses were removed with macroscopically clear margins (R0 and R1 resections) in four cases. In five patient cases adjuvant therapy was required. Three patients are still alive and free of disease. CONCLUSIONS: Retroperitoneal sarcomas present to be a therapeutic challenge based on their location, their extent at the time of diagnosis and the high risk of local recurrence or distant metastasis. Their management requires a multidisciplinary approach, with the surgical resection remaining the mainstay of curative treatment, combined with surveillance for early detection of recurrence or metastases.

Discarding IVF embryos: reporting on global practices.

PURPOSE: To provide a global scale report on a representative sample of the clinical embryology community depicting the practice of discarding supernumerary IVF embryos. METHODS: A web-based questionnaire titled "Anonymous questionnaire on embryo disposal practices" was designed in order to ensure anonymous participation of practicing clinical embryologists around the world. RESULTS: During a data collection period of 8 months, 703 filled-in questionnaires from 65 countries were acquired. According to the data acquired, the majority of practitioners, dispose of embryos by placing them directly in a trash can strictly dedicated for embryo disposal for both fresh and frozen cycles (39% and 36.7% respectively). Moreover, 66.4% of practitioners discard the embryos separately-case by case-at different time points during the day. Over half of embryologists (54%) wait until day 6 to discard the surplus embryos, while 65.5% do not implement a specially allocated incubator space as a designated waiting area prior to disposal. The majority of 63.1% reported that this is a witnessed procedure. The vast majority of embryologists (93%) do not employ different protocols for different groups of patients. Nonetheless, 17.8% reported the request to perform a ceremony for these embryos. Assessing the embryologists' perspective, 59.5% of participants stated that the embryology practice would benefit from a universally accepted and practiced protocol. CONCLUSION(S): This study uniquely provides insight into global embryo disposal practices and trends. Results highlight the divergence between reported practices, while indicating the significance on standardization of practice, with embryologists acknowledging the need for a universally accepted protocol implementation.

Intensive care unit outcomes following orthotopic liver transplantation: single-center experience and review of the literature.

BACKGROUND/AIM: Orthotopic Liver Transplantation (OLT) is the treatment of choice for patients with end stage liver disease, acute liver failure, hepatocellular carcinoma and metabolic disorders. As a result of improvement in surgical and anesthesiological skills, advanced understanding of transplant immunology and better critical care management of complications, patients survive longer after liver transplantation. It has been gradually achieved one-year survival rates of 80-90%. During the early post-operative period, all patients undergoing OLT are admitted to the intensive care unit, as they need a management of both preexisting patient's conditions and post-operative complications, usually due to either adverse intra-operative or post-operative events. The purpose of this review is the detailed recording, understanding and interpretation of immediate post-operative complications occurred in patients undergoing OLT, in intensive care unit. This could help to improve patient's treatment and reduce the incidence of complications, with further reduction of morbidity-mortality and cost. We also present our experience from the first 32 OLT patients from Liver Transplantation Unit of Laiko General Hospital, the only Liver Transplantation Unit in Athens. MATERIALS AND METHODS: This literature review was performed using the MEDLINE database. The key words were; Orthotopic liver transplantation; intensive care unit; post-operative complications; outcomes. One hundred-sixteen articles published in English until 2018 were used. We also use all the results from our 32 patients from our Liver Transplantation Unit during the period 07/2006 to 07/2009. RESULTS: All patients undergoing OLT admitted to the intensive care unit for a period of time, depending on the occurrence of post-operative complications. The incidence of primary failure ranges between 2-14%, whereas post-operative bleeding ranges between 7-15%. The treatment is usually conservative, although surgical repair may need in 10-15%. Acute renal failure post-operative is not an infrequent problem too, and has been reported to occur in 9% to 78% of cases. Acute rejection normally occurs 7-14 days after OLT. Additionally, the delay of the weaning from mechanical ventilation in the immediate post-operative period could increase the complications. Infectious complications are quite common almost from the first post-operative day in intensive care unit. CONCLUSIONS: Prolonged intensive care stay could increase the complications post-operative Infectious complications, renal and respiratory impairment are among the most common causes of early post-transplant morbidity and mortality.

Targeting histone deacetylases in endometrial cancer: a paradigm-shifting therapeutic strategy?

OBJECTIVE: Endometrial cancer is increasingly prevalent in western societies and affects mainly postmenopausal women; notably incidence rates have been rising by 1.9% per year on average since 2005. Although the early-stage endometrial cancer can be effectively managed with surgery, more advanced stages of the disease require multimodality treatment with varying results. In recent years, endometrial cancer has been extensively studied at the molecular level in an attempt to develop effective therapies. Recently, a family of compounds that alter epigenetic expression, namely histone deacetylase inhibitors, have shown promise as possible therapeutic agents in endometrial cancer. The present review aims to discuss the therapeutic potential of these agents. MATERIALS AND METHODS: This literature review was performed using the MEDLINE database; the search terms histone, deacetylase, inhibitors, endometrial, targeted therapies for endometrial cancer were employed to identify relevant studies. We only reviewed English language publications and also considered studies that were not entirely focused on endometrial cancer. Ultimately, sixty-four articles published until January 2018 were incorporated into our review. RESULTS: Studies in cell cultures have demonstrated that histone deacetylase inhibitors exert their antineoplastic activity by promoting expression of p21WAF1 and p27KIP1, cyclin-dependent kinase inhibitors, that have important roles in cell cycle regulation; importantly, the transcription of specific genes (e.g., E-cadherin, PTEN) that are commonly silenced in endometrial cancer is also enhanced. In addition to these abstracts effects, novel compounds with histone deacetylase inhibitor activity (e.g., scriptaid, trichostatin, entinostat) have also demonstrated significant antineoplastic activity both in vitro and in vivo, by liming tumor growth, inducing apoptosis, inhibiting angiogenesis and potentiating the effects of chemotherapy. CONCLUSIONS: The applications of histone deacetylase inhibitors in endometrial cancer appear promising; nonetheless, additional trials are necessary to establish the therapeutic role, clinical utility, and safety of these promising compounds.

Colonic casts: unexpected complications of colonic ischaemia.

Introduction Extensive colonic ischaemia can result in passage of a colonic 'cast' (CC) through the rectum. Case Study We report a 69-year-old male who initially underwent surgery to remove a sessile polyp. On postoperative day (POD)15, he was febrile, suffering from diarrhoea, and was treated conservatively. On POD18, the patient returned to our hospital with a CC that presented after defaecation. Computed tomography of the abdomen revealed a CC extending from the descending colon to the anal orifice with presentation of air between the affected colonic wall and the CC. The patient was treated conservatively and discharged on POD20 without complications having passed the CC (≈80cm) completely and becoming afebrile. Conclusions In most cases, the cause of CC passage is surgery for colorectal cancer or repair of an abdominal aortic aneurysm. A mild-to-severe presentation is dependent upon the bowel-wall layers affected by ischaemia and which therefore are included in the CC.

Incidence of inactive allele CYP2D6*4 among Greek women suffering from hormone-sensitive breast cancer.

BACKGROUND: The incidence of CYP2D6*4 among Caucasians is estimated up to 27%, while it is present in up to 90% of all poor metabolizers within the Caucasian population. The hypothesis under question is whether the presence of one or two non-functioning (null) alleles predicts an inferior outcome in postmenopausal women with breast cancer receiving adjuvant treatment with tamoxifen. The aim of the present study is to estimate the incidence of CYP2D6*4, in the Greek population and more precisely among females suffering from breast cancer. MATERIALS AND METHODS: Eighty unrelated mainland Greek female volunteers suffering from hormone-sensitive breast cancer were recruited during their primary handling or follow-up examination in order to provide samples for purification and polymerase chain reaction/restriction fragment length polymorphism (PCR-RFLP) of genomic DNA derived from buccal swabs. RESULTS: The incidence of individuals with at least one present allele*4 within the Hellenic population was estimated to be as high as 30% (n = 24/80), with a 95% confidence interval of 20% to 40%. From the statistical point of view, it can be securely stated that incidence of *4 among Greek women is over 20%. The incidence of homozygous carriers of *4 in the present sample occurred in 8.75%, while the incidence of allele*4 haplo-type occurred in 19.4% (n=160). CONCLUSION: Although the outcoming results for Greek women are actually in line with existing data for other European nations, it should be noted, that a routine CYP2D6 testing of women suffering from breast cancer is formally not recommended, as the clinical significance of CYP2D6 phenotype in treatment and outcome of breast cancer remains unclear.

Nonfunctioning symptomatic paraganglioma: Is there an optimal follow-up for patients with extra-adrenal benign paragangliomas.

Paragangliomas are rare neuroendocrine neoplasms originating from the embryological neural crest. In most cases, they exhibit a benign behavior. Here we report a case of a small symptomatic para-aortic paraganglioma, which was completely removed surgically and a review of the available literature regarding the optimal follow-up of a benign paraganglioma, since no guidelines are currently available for this rare entity.

Seasonal variation in breast cancer incidence. Circumstantial or default event?

UNLABELLED: It has been previously suggested that seasonality in the detection of breast cancer is mostly seen in countries with distinct climatic variations. Patient characteristics and delays have been implicated in the etiology of peak presentation. Seasonality has been more marked in premenopausal women, while delays have been attributed to both patients and health care systems. PATIENTS: A total of 1,411 women who presented with breast cancer to our department were analyzed according to their age, menopausal status, site, stage, grade, ER and PR status, c-erb-2 and Ki-67 (412) during the year. RESULTS: The seasonal variation was statistically significant, but no statistically significant differences were established between corresponding subgroups. CONCLUSION: The seasonal variation most probably reflects temporal, psychosocial and behavioral patterns in the Greek female population. Since we do not have the ability to recognize the actual onset of any cancer and then correlate it with various different independent factors we can not correlate its influence on survival or biological marker manifestations.

How can we treat a patient with liver cirrhosis (hepatitis C virus), hepatocellular carcinoma, and synchronous colon cancer?

INTRODUCTION: The coexistence of liver cirrhosis with hepatocellular carcinoma (HCC) and colon cancer (Ca), which is a rare clinical condition, was treated in a liver transplant recipient. PATIENTS AND METHODS: A 46-year-old man, diagnosed incidentally during an ultrasound (US) examination with a 3.5-cm HCC in segment VII related to chronic hepatitis C virus (HCV), was referred for liver resection. He underwent a laparoscopic protocol evaluation for liver cirrhosis. Liver appearance and biopsy of the left lobe showed Child B/C liver cirrhosis. Because he fulfilled the Milan criteria, we suggested an orthotopic liver transplantation (OLT). During protocol colonoscopy, we discovered an ulcerative sigmoid colon Ca. Three weeks after completing the pre-OLT assessment he underwent an OLT and was discharged home on day 9 on an immunosuppressive regimen of Everolimus, Myfortic, and Prezolone. Two months after transplantation, the patient underwent a sigmoidectomy and for nearly 1 month thereafter received chemotherapy for colon Ca (6 cycles of FOLFOX:Folinic Acid+Fluorouracil+Oxaliplatin). One and a half years after OLT, patient was in good condition but presented with an increased alpha fetoprotein (a-FP) without other findings. A couple of months later we discovered a colon Ca recurrence and 3 small liver metastases. Patient underwent a bowel resection with Hartmann's procedure. Almost immediately after the last operation, he was found to suffer multiple myeloma. He underwent chemotherapy for both malignancies with good responses, but a few months later died of severe sepsis. DISCUSSION: The relevant literature regarding treatment of liver cirrhosis complicated with HCC and synchronous colon Ca reveals poor and controversial outcomes. Our patient underwent chemotherapy immediately after colon resection in the presence of with a good functioning liver. Although his condition was satisfactory after OLT, the optimal treatment of such complicated patients is as yet uncertain.

Primary malignant fibrous histiocytoma of the spleen--an extremely rare entity.

Malignant fibrous histiocytoma (MFH) is a frequently occurring soft tissue tumour with aggressive biological behaviour. Primary MFH of the spleen, however, is an exceedingly rare occurrence. Herein, we report a case of such a neoplasm in a 66-year-old female. The tumour was well localised within the spleen and the patient remains alive and well 34 months after splenectomy without any adjuvant therapy. A brief discussion and review of the few cases of primary splenic MFH previously reported in the English language literature is included.

Impact of breast cancer surgery on the self-esteem and sexual life of female patients.

Patient satisfaction with cosmetic outcome and the psychological impact of breast cancer surgery were evaluated. A total of 207 patients with primary breast cancer, treated with either breast-conserving surgery (n = 83), modified radical mastectomy without reconstruction (n = 108), or mastectomy with delayed breast reconstruction (n = 16) rated their cosmetic outcome and satisfaction following surgery, and the impact of surgery on their self-esteem and sexual life, by questionnaire. Patients undergoing breast-conserving surgery were most satisfied with their surgery and body image, followed by those treated with mastectomy with delayed reconstruction. Although diagnosis of breast cancer had a negative impact on the psychology of all patients, those undergoing breast-conserving surgery or mastectomy with delayed reconstruction were more satisfied and reported a lower impact on their self-esteem and sexual life versus those who only had mastectomy. Diagnosis of breast cancer has a negative psychological impact on the patient, but the type of surgery has a significant role in post-operative self-esteem and sexual life.

Adrenal rupture after blunt abdominal trauma.

The case of a patient, victim of a car accident, with rupture of the left adrenal gland associated with splenic rupture and liver laceration is reported. Injury of the adrenal glands is most frequently caused by blunt abdominal trauma and is usually associated with injuries of other abdominal organs. Careful exploration of the upper retroperitoneal space where adrenals are located is considered necessary during laparotomy for severe blunt abdominal trauma. If adrenal lesion has been overlooked continuous hemorrhage may occur, which could be lethal if not promptly treated.

Crossover analysis using immunofluorescent detection of MLH1 foci in frozen-thawed testicular tissue.

To date, the effects of freezing on spermatogenesis have not yet been fully investigated at a molecular level. Antibody localization studies have identified the MutL homolog 1 (MLH1) protein, a mis-match repair protein, at the prophase I stage of meiosis, which allows the detection of recombination foci during pachytene. This study investigated the effect of long-term testicular tissue cryopreservation on meiotic prophase I, identified by recombination foci frequency and synaptonemal complex (SC) integrity. Frozen-thawed testicular tissues from 12 males who had each fathered a child were used. Because vasectomy or reverse vasectomy procedures are rare in the locale of the investigation, it was not possible to obtain fresh testicular tissue and use the males as their own controls. Immunocytogenetic analysis of 612 spermatocytes at the pachytene stage was performed. The results indicated a mean number of MLH1 foci of 49.2 (SD +/- 5.9), and no correlation was found between the freezing period, the MLH1 frequency and the SC integrity. The results suggest that freezing of testicular tissue taken post-puberty does not appear to be detrimental to the crossover process as identified by occurrence of MLH1 loci.

Evaluation of sperm chromatin quality and screening of Y chromosome microdeletions in Greek males with severe oligozoospermia.

Normal (n = 20) and abnormal (n = 21) semen samples were explored for possible relationships between conventional semen parameters, chromatin status, and microdeletions in the Y chromosome. DNA fragmentation was detected by the terminal deoxynucleotidyl trasferase-mediated dUTP-nick end labelling (TUNEL) assay, chromatin condensation, and DNA packaging quality were assessed by chromomycin A3 (CMA(3)) staining. All men were investigated for Y chromosome microdeletions using polymerase chain reaction (PCR). No deletions were detected in 21 severely oligozoospermic men for the three screened regions (AZFa, AZFb, and AZFc). Men with normal semen parameters showed better chromatin condensation. Spermatozoa with low motility were more likely to contain loosely packaged chromatin. In the abnormal semen group, DNA fragmentation (TUNEL) correlated significantly with sperm motility, concentration, and chromatin packaging assessed by chromomycin A3. However sperm morphology did not correlate significantly with TUNEL and CMA(3) staining.

Wire localisation biopsy of non-palpable breast lesions: reasons for unsuccessful excision.

Surgical excision following needle-wire localization of nonpalpable, mammographically detected breast lesions is a very valuable diagnostic and therapeutic procedure. No further treatment is usually required after establishing an accurate histological benign diagnosis of indeterminate lesions on preoperative assessment. On the other hand, ductal carcinoma in-situ (DCIS) and early invasive cancer, properly excised, may sometimes require further management depending on specific histologic findings. An uncommon problem of this procedure is the failure to identify, localize or excise the breast lesion. In this review article, factors that contribute to the failed needle localization procedure are presented.

Surgical management of nipple discharge.

PURPOSE: Nipple discharge is reported in 2.5-3% of women with breast carcinoma. Breast carcinoma is found in approximately 8% of surgically treated patients presenting with bloody nipple discharge (ND). METHODS: In the present study 110 women with ND as a presenting symptom were examined. The discharge was spontaneous in 76 and elicited in 11 patients. RESULTS: After surgical intervention benign breast disease was found in 85% of patients. Thirteen women (15%) were found to have malignancy and underwent additional surgery. Cytology of the discharge was positive or suspicious for malignancy in only seven out of 13 patients found to have in situ or invasive carcinoma. CONCLUSION: Women with spontaneous, single duct ND, especially when it is darkish or bloody, should have cytological examination of the fluid and mammography according to their age or additional clinical findings. Most of them will require a microdochectomy, as the possibility of finding a carcinoma among those women is between 10-15%. However, single duct papilloma is the most common cause of bloody discharge.

Paraffinomas of the breast or oleogranulomatous mastitis-a rare entity.

Breast augmentation with the injection of a high-viscosity fluid has been practiced in the past, especially in Asia, resulting in most cases in different varieties of paraffinoma, alternatively known as oleogranulomatous mastitis, a complication of a 'foreign body' reaction type. We report our series of four consecutive cases of destructive paraffinomas that occurred in female patients who had had paraffin injection for cosmetic reasons. Complications due to paraffinoma reactions of breast tissue and skin, as well as progression of the disease and treatment of our patients, are presented here, together with a review of the relevant literature.

Evaluation of gram stain as an alternative in the assessment of human spermatozoa quality.

INTRODUCTION: During spermiogenesis, protaminosis and sperm chromatin condensation are important prerequisites for the preservation of DNA integrity in spermatozoa. The aim of this study is to assess Gram stain as an alternative technique for the evaluation of human sperm chromatin condensation status. PATIENTS AND METHODS: Aniline blue and Gram staining were applied to semen samples from 34 donors in order to determine the relationship between sperm chromatin condensation and infertility. In addition, the possible correlation between morphology and vitality (eosin-Y staining) of spermatozoa compared with their nuclear status (aniline blue and Gram staining) was studied. RESULTS: Chromatin condensation and sperm vitality were significantly higher in fertile men compared to the subfertile. A significant correlation was found between chromatin condensation and (a) sperm vitality (p < 0.01), and (b) nuclear protein status (p < 0.01). CONCLUSIONS: Gram staining may be used as a routine method in assisted reproduction laboratories and could assist in the evaluation of sperm quality as well as in the selection of the appropriate fertilization technique.