Digital glomus tumors: a 29-year experience with a lateral subperiosteal approach

The excision of distal digital glomus tumors has traditionally been performed directly over the involved nail bed. This can lead to nail deformities that are often unacceptable for the surgeon and the patient. The authors describe their experience with successful excision of digital glomus tumors using a lateral subperiosteal approach, which creates a dorsal flap. In 29 years, 19 patients were diagnosed with digital glomus tumors. All patients underwent excision using the lateral subperiosteal approach. The mean tumor size was 0.52 cm. The tumors were located on the pulp of the distal phalanx in two patients (10.5 percent) and subungually in 17 patients (89.5 percent). In all patients, preoperative clinical diagnosis was confirmed postoperatively with the biopsy result. Complications occurred in only two patients and included one paronychia and one temporary nail loss. The overall recurrence rate was 15.7 percent. All patients remained asymptomatic after surgery and regained full active and passive range of motion. There were no nail deformities by this approach. This technique represents a safe and effective approach to excising digital glomus tumors.     

Equine Sarcoids. A Clinical and Epidemiological Study in Relation to Equine Leucocyte Antigens (ELA)

Associations between clinical parameters of sarcoids and the equine leucocyte antigen system (ELA) were analysed for 120 Swedish horses. Median age of affected horses was 5.2 years, and the majority presented with solitary tumors between 2 and 5 cm in diameter and ventral abdomen was a predilection site. Clinical signs first appeared at a median age of 3.5 years, and sarcoids at different locations first appeared at different ages. Lesions at different sites differed in size, and multiple tumors, early onset, long duration, and older age all had an association with large size. Clinical manifestations of sarcoids and the association between certain ELA-specificities and early onset (A5) and increased recurrence rates after surgery (W13), in addition to increased prevalence (A3W13), strengthen further that some horses are inherently predisposed to sarcoid growth. Unassociated with any clinical parameters, one third of the untreated horses became free of sarcoids due to “spontaneous” regression, perhaps as a result of immune responses against the tumors. Seventy percent of the horses were treated (mostly by excision), and large size was the main parameter promoting treatment. Excision had no significant effect on possibly remaining sarcoids. Recurrence rate after first treatment was about 35%, with the majority of tumors recurring within 4 months. Early onset, long duration, large size, and localization to distal limbs all appeared to increase risk of recurrence. Early treatment, performed under general anesthesia in recumbency which permits wide excision and measures to avoid autoinoculation, significantly reduced recurrence rates.     

Management of Patients With Low-Risk Papillary Thyroid Carcinoma

ObjectiveTo define a rational, cost-effective, simple approach to managing most patients with papillary thyroid cancer (PTC) who are at low-risk of either cause-specific mortality or tumor recurrence.MethodsTaking advantage of the collective experience of a cohort of 2512 patients with PTC who had initial definitive treatment at the Mayo Clinic in Rochester, Minnesota, between 1940 and 2000, a 5-step approach to the management of low-risk PTC has been devised. This program is based on appropriate preoperative ultrasound localization of neck disease and potentially curative surgery consisting of near-total or total thyroidectomy, with appropriate neck nodal exploration and resection.ResultsThe emphasis of the present program is on the extent of initial surgery, where optimal care is ascribed to a near-total thyroidectomy with curative intent and appropriate neck nodal resection as predicated by appropriate preoperative ultrasonography evaluation of regional lymph nodes. Radioiodine remnant ablation (RRA) is not applicable to patients with PTC who are defined on the day of definitive initial surgery to be at low risk as defined by a metastasis, age, completeness of resection, invasion, and size (MACIS) score of less than 6.ConclusionThe outlook for patients with low-risk PTC is very optimistic, with rates at 30 postoperative years of only 1% for cause-specific mortality and less than 15% for tumor recurrence at any site. The long-term results obtained by potentially curative bilateral resection, appropriate regional lymph nodal excision, and selective use of RRA are excellent. Realistically improving these acceptably low rates for cause-specific mortality and tumor recurrence may be difficult. (Endocr Pract. 2007;13:521-533)     

Managing soft tissue sarcomas in a developing country: are prognostic factors similar to those of developed world?

ABSTRACT: BACKGROUND: Managing soft tissue sarcomas (STS) in a developing country with limited financial resources and a poor health referral system is a challenge. Presenting late, these extremity STS are prone to recurrence despite apparently complete resection. This study aimed to explore and compare the impact of clinico-pathological factors on recurrence and survival in Pakistan with the corresponding figures quoted from the developed world. METHODS: An institutional review was performed on all patients with primary STS of the extremities operated on between 1994 and 2008. The prognostic influence of clinical, pathologic, and treatment variables on local recurrence free survival (LRFS), metastasis free survival (MFS) and overall survival (OS) were analyzed by univariate and multivariate Cox regression analysis and Kaplan Meier survival curves. RESULTS: A total of 84 patients with a mean age of 41.8 +/- 21.9 years were included in the study. The local recurrence rate was 14.3% after a median of 6 (mean 7.4) months. Metastases occurred in 7 patients (8.3%) and 65 patients were alive without evidence of disease after a mean follow-up of 52.6 +/- 39.8 months. Tumor size > 5 cm, grade 3 tumors and margin < 10 mm significantly increased local recurrence rates. A margin >= 10 mm and age < 45 years significantly enhanced cumulative survival. Significant multivariate risk factors for metastases were margin < 10 mm and tumor grade G3. CONCLUSIONS: Despite a poor health referral system in our country, our results are no different from those reported from the developed world. Surgical margins and tumor grade prognostically influenced LRFS, MFS and OS.     

MIB-I as a proliferative activity marker of the multiform glioblastomas

MIB-I is a proliferative activity marker of multiform glioblastomas which are the most frequent tumors of the central nervous system. They are characterizad by differential rate and prognosis. The aim of the study was to determine the proliferative activity of multiform glioblastomas and estimation of the correlation between tumors' proliferative activity and tumors' localization, size, patients' age and sex. 24 patients (18 females and 6 males) with multiform glioblastomas were analyzed. The mean patients' age was 52.1. The proliferative activity was calculated as a proliferation index: IP for MIB-I. Cells with positive reaction were determined by MIB-I which was compared to all neoplastic cells. The most frequent localization of the tumors were frontal and temporal lobes of the brain. The size of the tumors ranged from 2.5 to 5.3 cm (mean 3.9). Mean IP was 43.2 (SD+/-17.4). We found no correlation between IP MIB-I and localization of the tumor, patients' age and sex. There was a marginal statistically significant correlation between IP MIB-I and size of the tumor (p=0.005).     

DNA ploidy in soft tissue sarcoma: comparison of flow and image cytometry with clinical follow-up in 93 patients

In soft tissue sarcoma, the prognostic importance of DNA ploidy status is limited. One possible explanation may be technical; small non-diploid stemlines will be diluted in relation to the presence of normal diploid cells and may not be detected by flow cytometry (FCM). We assessed DNA ploidy status in 93 tumors with both FCM and image cytometry (ICM). ICM may permit the exclusion of non-relevant cells. The ability of the two methods to detect non-diploid stemlines was compared, as were the prognostic consequences. The patients (54 males) had a median age of 69 years. Surgical procedures were performed on all patients. None of the patients had received preoperative radiotherapy or chemotherapy. FCM and ICM were performed with standard methods. The prognostic value was assessed with univariate and multivariate analysis. In 82 of the 93 tumors, a concordant ploidy status by FCM and ICM was found. In 5 FCM type 1-2 tumors (diploid), the identification of non-diploid stemlines by ICM did not influence the metastatic rates. Increasing tumor size, histotype other than liposarcoma, increasing malignancy grade, tumor necrosis, and ICM non-diploidy were univariate prognostic factors for metastasis. In a multivariate analysis, only tumor size larger than 9 cm was a prognostic factor. In about 10% of the tumors, a discrepancy between FCM and ICM ploidy status was found, but we could not find a consistent prognostic consequence of this. Neither FCM nor ICM ploidy status was an independent prognostic factor.     

原发性肠系膜肿瘤的诊断与治疗

目的:探讨原发性肠系膜肿瘤的诊断及治疗方法。方法:对各种类型的原发性肠系膜肿瘤的临床表现、术前检查及诊断、治疗等进行对比分析。结果:原发性肠系膜肿瘤临床表现多样、复杂,缺乏特异性诊断依据,但在B超引导下细针穿刺等有助于其诊断。实性肿瘤多为恶性,手术切除率低。囊性肿瘤多为良性,手术切除率高。结论:手术是治疗本病的唯一有效手段。     

Radiographic analysis of growth in pediatric microsurgical toe-to-hand transfers

Microsurgical toe-to-hand transfers may provide improved hand function in children with absent digits. To date, documentation of the growth potential of these transferred digits has not been performed. This study reviewed the authors' series of pediatric toe-to-hand transfers, with specific attention paid to measuring growth by radiographic analysis. From 1995 to 2000, 23 toe-to-hand transfers were performed in 18 children. Age at time of transfer ranged from 2.8 to 13 years. Indications included constriction band syndrome, transverse deficiency, longitudinal deficiency, traumatic amputation, and vascular malformation. The transfers were successful in 22 of 23 procedures (96 percent success rate). Radiographic analysis of growth was performed using three criteria: (1) appearance of open epiphyseal plates, (2) comparison with preoperative radiographs, and (3) comparison with radiographs of the contralateral control toe. Epiphyseal plates remained open on postoperative radiographs in 27 of 28 phalanges (96 percent) at a mean of 12 months' follow-up (range, 1 to 36 months). The preoperative foot radiographs were compared with serial radiographs of the transferred toe over time. In 10 toe transfers with follow-up greater than 6 months (mean, 21 months), nine patients had increased bony length in the transferred digit. In four patients, radiographs of the toe transfer were compared with radiographs of the corresponding toe on the opposite foot. With a mean follow-up of 29 months, all patients had equal length measurements of the toe transfer with the contralateral toe control. These data provide objective evidence that digital growth potential is preserved in toe-to-hand transfers. Furthermore, this bone growth is comparable with that of the corresponding toe on the contralateral foot. Therefore, microsurgical toe-to-hand transfers may provide children with extra digits that maintain growth and improve hand function.     

Histopathological study of breast cancer and normal breast tissue after magnetic resonance-guided cryotherapy ablation

BACKGROUND: Cryotherapy ablation is a minimally invasive procedure being investigated as an alternative to conventional surgery. There are few reports in breast cancer. AIM: Evaluate the histopathology of tumoral and normal breast tissue after cryotherapy. METHODS: Eleven patients with clinically <2.0cm and ultrasound visible tumors were studied. Invasive carcinoma was documented by preoperative mammography, magnetic resonance imaging and biopsies. Cryotherapy needles were inserted in the tumor under magnetic resonance guidance and deep freezed with a CRYO-HIT TM System-3. Lumpectomy was performed within 4-5 weeks following cryoablation and submitted for pathological examination including immunostaining of keratins. RESULTS: The tumoral response after cryoablation was variable. In 4 cases there was no viable invasive carcinoma left and focal DCIS only in 2. In 6 cases, residual invasive carcinoma of various size was present with DCIS inside or outside the cryozone. One case could not be evaluated because the cryozone was adjacent to the tumor due to technical problems. Histologically, the normal breast parenchyma of the cryozone showed dense fibrosis, fat necrosis, xanthogranulomatous reaction, endovascular fibrosis and haemorrhages in all cases. The positive immunostaining of keratins revealed remnants of cytoskeleton of carcinomatous cells in the necrotic areas without any viable tumoral cells on routine stains. Skin ulceration and/or necrosis were observed in five patients. CONCLUSIONS: Cryotherapy allows tumor destruction of variable extent in breast carcinomas <2.0cm in diameter. Immunostaining of keratins is useful to identify cytoskeleton remnants of tumor cells in devitalized areas.     

Surgical treatment of endocrine tumors of the pancreas

Surgeons have a key role in the management of most endocrine tumors of the pancreas. The objective of surgery is either curative or palliative, according to the resectability of the tumor. The preoperative management includes the assertion of the tumoral syndrome, the localization of the tumor by imaging techniques and the preparation of the patient to surgery. Emergency indications to operate have become exceptional since the temporary control of inappropriate secretions by pharmacologic agents is available. Intraoperative ultrasonography is useful both for detection of the tumors and selection of the best procedure for resection. A careful postoperative follow-up is advocated for patients with malignant tumors and MEN-1, since iterative resections can be undertaken in case of limited tumoral recurrence or liver metastases. The place of hepatic transplantation for diffuse metastases is still under evaluation.     

Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution

Background and purpose

Pediatric desmoid tumor (PDT) is rare and has a high local recurrence rate. The purpose of the present study was to analyze clinical risk factors of local recurrence in PDT patients.

Materials and methods

We reviewed clinical data of 66 PDT patients from 2004 to 2015. All patients underwent macroscopically complete resection, and some recurrent tumors were prescribed radiotherapy. Factors such as sex, age at presentation, location, and proximity to nerves or vasculature were analyzed. The local recurrence rate and recurrence-free survival were analyzed with these factors.

Results

All patients in the present study were children and had extra-abdominal tumors. The median follow-up time was 6.6?years. Thirty-six (55%) patients had local recurrence. Age, sex, tumor site, tumor size, and proximity to nerves/vasculature had a significant impact on prognosis in univariate analysis. Radiotherapy decreased the local recurrence rate. In multivariate analysis, younger age, tumor location in buttocks, larger tumor, and proximity to important nerves/vasculature were independent risk factors for poor prognosis.

Conclusions

Favorable therapeutic strategies could be selected according to the preoperative prognostic risk factors. Radiotherapy should be considered for local recurrence of PDT.

     

Endoscopic Ultrasonography - A Sensitive Tool in the Preoperative Localization of Insulinoma

ObjectiveA number of imaging modalities have been used in the preoperative localization of insulinomas. Computed tomography (CT) is the most commonly employed modality. Endoscopic ultrasound (EUS) allows the transducer to be placed in close proximity to the pancreas, thereby yielding higher quality images, which facilitates accurate localization, minimally invasive surgery, and a lower occurrence of residual tumors, all of which contribute to a better clinical outcome.MethodsWe analyzed the hospital records of all adult patients (age >18 years) diagnosed with insulinoma between October 2004 and September 2010. The diagnosis was based on the clinical practice guidelines of the American Endocrine Society. We compared the sensitivities of EUS and multidetector computed tomography (MDCT) in lesion.ResultsEighteen patients were seen over a period of 6 years, and all underwent EUS. MDCT scans were carried out in 17 patients. EUS had greater sensitivity (89%) in localizing insulinomas compared to CT (69%). In this series, the lesions that were missed on CT but picked up on EUS were smaller (<12 mm, P<.001). Lesions that were near mesenteric vessels and those located in the head of the pancreas were more likely to be missed on CT.ConclusionsEUS has a greater sensitivity in identifying and localizing insulinomas. As availability increases, EUS should be part of a preoperative insulinoma workup. (Endocr Pract. 2013;19:602-608)     

Impact of protein stability, cellular localization, and abundance on proteomic detection of tumor-derived proteins in plasma

Tumor-derived, circulating proteins are potentially useful as biomarkers for detection of cancer, for monitoring of disease progression, regression and recurrence, and for assessment of therapeutic response. Here we interrogated how a protein's stability, cellular localization, and abundance affect its observability in blood by mass-spectrometry-based proteomics techniques. We performed proteomic profiling on tumors and plasma from two different xenograft mouse models. A statistical analysis of this data revealed protein properties indicative of the detection level in plasma. Though 20% of the proteins identified in plasma were tumor-derived, only 5% of the proteins observed in the tumor tissue were found in plasma. Both intracellular and extracellular tumor proteins were observed in plasma; however, after normalizing for tumor abundance, extracellular proteins were seven times more likely to be detected. Although proteins that were more abundant in the tumor were also more likely to be observed in plasma, the relationship was nonlinear: Doubling the spectral count increased detection rate by only 50%. Many secreted proteins, even those with relatively low spectral count, were observed in plasma, but few low abundance intracellular proteins were observed. Proteins predicted to be stable by dipeptide composition were significantly more likely to be identified in plasma than less stable proteins. The number of tryptic peptides in a protein was not significantly related to the chance of a protein being observed in plasma. Quantitative comparison of large versus small tumors revealed that the abundance of proteins in plasma as measured by spectral count was associated with the tumor size, but the relationship was not one-to-one; a 3-fold decrease in tumor size resulted in a 16-fold decrease in protein abundance in plasma. This study provides quantitative support for a tumor-derived marker prioritization strategy that favors secreted and stable proteins over all but the most abundant intracellular proteins.     

乳腺癌改良根治术患者术后复发转移的危险因素及血清CA125、COX-2、sTNFR-P55的预测价值研究

摘要 目的：探讨乳腺癌改良根治术患者术后复发转移的危险因素及血清糖类抗原125（CA125）、环加氧酶-2（COX-2）、可溶性肿瘤坏死因子受体P55（sTNFR-P55）的预测价值。方法：对2014年1月至2016年12月新疆医科大学第一附属医院收治的109例行乳腺癌改良根治术的乳腺癌患者进行前瞻性研究,所有患者术后均随访5年,其中2例失访,107例完成随访。根据5年内患者复发转移情况将其分为复发转移组（n=31）和未复发转移组（n=76）。收集患者入院时的临床病理资料,采用电化学发光法检测术前血清CA125,采用酶联免疫吸附法检测术前血清COX-2、sTNFR-P55。采用logistic回归模型分析患者术后复发转移的影响因素,绘制受试者工作特征（ROC）曲线评估血清CA125、COX-2、sTNFR-P55对术后复发转移的预测价值。结果：复发转移组肿瘤直径＞5 cm、浸润性非特殊癌、脉管癌栓、雌激素受体（ER）/孕激素受体（PR）阴性、无内分泌治疗构成比、TNM分期IIIA期、腋窝淋巴结转移数量4~9个构成比高于未复发转移组（P<0.05）。复发转移组血清CA125、COX-2、sTNFR-P55水平高于未复发转移组（P<0.05）。多因素logistic回归分析结果显示,肿瘤直径＞5 cm、浸润性非特殊癌、TNM分期IIIA期、脉管癌栓、腋窝淋巴结转移数量4~9个、CA125升高、COX-2升高、sTNFR-P55升高是乳腺癌改良根治术患者术后5年内复发转移的独立危险因素（OR=1.318、1.213、1.223、1.137、1.257、1.241、1.313、1.351,P<0.05）。血清CA125、COX-2、sTNFR-P55均可有效预测乳腺癌术后复发转移,曲线下面积（AUC）分别为0.803、0.749、0.761,三指标联合预测术后复发转移的AUC为0.915,灵敏度和特异度分别为0.94、0.83。结论：肿瘤直径、浸润性非特殊癌、TNM分期、脉管癌栓、腋窝淋巴结转移数量以及术前血清CA125、COX-2、sTNFR-P55异常升高是乳腺癌改良根治术患者术后5年内复发转移的危险因素,术前血清CA125、COX-2、sTNFR-P55联合检测可预测乳腺癌改良根治术后的复发转移风险。     

TURis-Bt术前膀胱灌注表柔比星治疗NMIUC的临床疗效观察

目的:探讨TURis-Bt术前膀胱灌注表柔比星治疗非肌层浸润性膀胱癌(NMIUC)的临床疗效。方法:选取2008年9月至2012年1月潍坊市中医院和上海交通大学附属第一人民医院泌尿外科收治的76例NMIUC患者,并将其随机分为观察组(TURis-Bt术前膀胱灌注表柔比星+术后常规灌注组)41例和对照组(TURis-Bt术后常规表柔比星膀胱灌注组)35例。灌注前将50mg表柔比星溶解于50ml 5%葡萄糖注射液,术前膀胱保留灌注30分钟后膀胱镜观察肿瘤组织及周围膀胱粘膜染色情况,将表柔比星橙染的膀胱粘膜活检并行TURis-Bt;对照组取瘤旁2 cm处及其他部位膀胱粘膜多点活检。比较两组的原位癌(CIS)、非典型性增生及腺性膀胱炎等病变检出率和术后肿瘤的复发率。结果:观察组患者瘤旁膀胱粘膜橙染56处,其中7处病理证实为膀胱原位癌、5处为非典型性增生、11处为腺性膀胱炎;对照组患者膀胱原位癌1处、非典型性增生3处、腺性膀胱炎2处,两组病变阳性率分别为41.1%(23/56)和13.4%(17/127),差异有显著统计学意义(P0.01)。观察组与对照组术后2年内肿瘤复发率分别为10.3%(4/39)和35.3%(12/34),差异有统计学意义(P0.05)。结论:TURis-Bt术前膀胱灌注表柔比星能提高NMIUC病变的早期检出率并降低肿瘤术后复发率。     

Establishment of a Lung Metastatic Breast Tumor Xenograft Model in Nude Rats

Objective

Larger animal models provide relevant tumor burden in the development of advanced clinical imaging methods for non-invasive cancer detection and diagnosis, and are especially valuable for studying metastatic disease. Most available experimental models, however, are based on immune-compromised mice. To lay the foundation for studying spontaneous metastasis using non-invasive magnetic resonance imaging (MRI), this study aims to establish a highly metastatic breast cancer xenograft model in nude rats.

Materials and Methods

A highly metastatic variant of human adenocarcinoma MDA-MB-231 known as LM2 was inoculated into nude rats. Orthotopic and subcutaneous (flank) sites were compared, with half of the orthotopic injections guided by ultrasound imaging. MRI with gadolinium contrast administration was performed weekly beginning on Day 6 and ending on Day 104. Excised tumors were assessed on histology using hematoxylin and eosin and CD34. Fisher''s exact test was used to compare successful tumor induction amongst different inoculation methods.

Results

Primary LM2 tumors were established orthotopically in all cases under ultrasound-guided injection, and none otherwise (p = 0.0028). Contrast-enhanced MRI revealed rapidly progressing tumors that reached critical size (15 mm diameter) in 2 to 3 weeks after inoculation. MRI and histology findings were consistent: LM2 tumors were characterized by low vascularity confined to the tumor rim and large necrotic cores with increasing interstitial fluid pressure.

Conclusions

The metastatic LM2 breast tumor model was successfully established in the mammary fat pads of nude rats, using ultrasound needle guidance as a non-invasive alternative to surgery. This platform lays the foundation for future development and application of MRI to study spontaneous metastasis and different stages throughout the metastatic cascade.     

Local recurrence risk after skin-sparing and conventional mastectomy: a 6-year follow-up

In this study, the records of all patients at the University of Texas M. D. Anderson Cancer Center with T1 or T2 breast cancer who were treated between March of 1986 and November of 1990 with mastectomy followed by immediate breast reconstruction were reviewed for the presence of recurrent disease. Patients with in situ disease were not included. Patients were included in the study if a local recurrence occurred (regardless of the length of follow-up) or if a follow-up of 6 years or longer could be obtained. Patients were grouped according to the use or nonuse of skin-sparing mastectomy, by tumor stage, and by nuclear grade of the tumor. The series included 154 patients, of whom 114 had skin-sparing mastectomies and 40 had nonskin-sparing mastectomies. The local recurrence rate in the skin-sparing mastectomy group was 7.0 percent, whereas in the nonskin-sparing mastectomy group it was 7.5 percent. The sample size in the nonskin-sparing mastectomy group was too small for meaningful statistical analysis, but the data suggest that there is no clinically important difference in recurrence rates between the two groups. We conclude that the use of skin-sparing technique for early breast cancer patients does not significantly increase the risk of tumor recurrence after mastectomy.     

Neuroendocrine tumors of gastrointestinal tract in own material

Neuroendocrine tumors are rare gastrointestinal tract disorders, in which diagnosis and treatment are often difficult. The aim of the paper is to present two cases of patients with neuroendocrine tumor of gastrointestinal tract, who underwent surgical procedure in II Department of General and Gastroenterological Surgery of Medical University of Bia?ystok in 2005. A 63-year-old female patient with primary diagnosis of neuroendicrine tumor metastases in liver was not successfully investigated for primary tumor in the preoperative period. The laparotomy procedure indicated the malignant neuroendocrine tumor in the terminal ileum and metastases to the liver and to the greater omentum. The right hemicolectomy and liver metastatic segment VII and VIII resection were performed. The neurological disturbances of obscure origin were observed in the postoperative period and the patient suddenly died on the 15(th) day after surgery. A 57-years-old male patient was operated on for lymph node recurrence of gastric tumor. Pathologic examination of tissue sample revealed the diagnosis of carcinoid. The patient underwent subtotal gastric resection for a pyloric ulcer, diagnosed as Adenocarcinoma G2 pT2N0M0 6 years before. Liver and abdominal node metastases, confirmed by octreoscan, were observed after lymphadenectomy, The treatment of somatostatin analogues (LAR octreotide) was used. In spite of therapy the patient died. The authors present their own experiences and show the preoperative diagnostic difficulties in patients with neuroendocrine gastrointestinal tumors. Unexpected neurological complications in the treatment course were described.     

Diagnostic and therapeutic difficulties in MEN 1 syndrome

MEN 1 syndrome (Multiple Endocrine Neoplasia type 1) is a rare endocrine disorder characterized by the association of tumors in several endocrine glands, mainly in parathyroids, gut and pituitary. At our institution in the years 1982-2004 we have followed 26 patients with MEN 1 syndrome belonging to 19 families. The diagnosis of MEN 1 was based on Gubbio Consensus (JCEM 86: 5658-5671, 2001). Mean age at the diagnosis of MEN 1 was 35 years. Primary hyperparathyroidism was the most frequent pathology, which was diagnosed in 25 of 26 patients (96%). Gut endocrine tumors were found in 20 patients (77%), while pituitary tumors in 18 (70%). Non-functioning gut tumors were most frequent (n=9), followed by insulinoma (n=7) and gastrinoma (n=4). Prolactinoma was the most frequent pituitary tumor found in 12 patients (67%). Three patients died during the observation period - all of them of generalized gut endocrine tumor (gastrinoma in 2 cases and foregut carcinoid in one case). The management of MEN 1 is not easy and careful analysis of clinical picture is necessary in each individual case. Several important observations can be made on the basis of own experience and the literature: 1. In each sporadic pathology, which may be a part of MEN 1, one should consider. the possibility of MEN 1. The individual MEN 1 abnormalities are often diagnosed after 40 and later 2. MEN 1 tumor are usually multiple thus necessitating a different therapeutic approach (more radical surgery) 3. The most valuable screening tests are: Ca++, PP, CgA and prolactin 4. Endoscopic ultrasound is the most specific method for the localization of pancreatic endocrine tumors. 5. The results of surgical treatment of MEN 1 tumors are worse than that of sporadic tumors. 6. Prognosis in MEN 1 is determined by the behaviour of gut neuroendocrine tumor 7. No genotype/phenotype correlation in MEN 1 syndrome was found so far. In summary, it should be underlined that MEN 1 syndrome is an endocrine disorder, in which early diagnosis and optimal treatment may significantly improve the prognosis.     

DNA cytophotometric analysis of breast cancer. Follow-up for 10 years.

Forty-four cases of newly diagnosed invasive ductal breast cancer were studied by static cytometry at the time of diagnosis and were followed for 10 years to determine if the survival rate correlated with the nuclear DNA measurements. In 22 cases the stem line was in the 2c range, in 15 cases in the 3c range and in 7 cases in the 4c range. Thirty-four percent (n = 15) of the patients died of metastases during the first five years, 11% (n = 5) died of metastases between 5 and 10 years, and 55% (n = 24) survived for > 10 years without metastases. No correlation between increased tumor cell proliferation and recurrence of tumors could be found. In this study all patients with diploid tumors, tumor size T1 and negative lymph node status survived for > 10 years. In the presence of node metastasis (T1N1M0) all patients (n = 4) having tumors with the stem line in the 2c range survived for 5 years but only 2/4 for > 10 years. The percentage of patients surviving for 10 years showed no significant differences between the groups: in the 2c range, 59% (13/22); in the 3c range, 53% (8/15); and in the 4c range, 43% (3/7). The small number of cases does not allow definitive statistical conclusions, although the logistic regression analyses showed that stem line, pT and pN were the important prognostic factors for five-year survival. However, only pT and especially pN were of prognostic value for 10-year survival.