Osseous metastases of uterine leiomyosarcoma: Analysis of survival & surgical management.

BACKGROUND: Uterine leiomyosarcoma represents a seldom-encountered subset within the spectrum of uterine malignancies. Occurrences of appendicular skeletal metastases in uterine leiomyosarcomas are infrequent. In this study, we examined patient surveys to elucidate the clinical characteristics and outcomes of individuals with uterine leiomyosarcoma exhibiting metastatic dissemination to these anatomical regions. We hypothesized that palliative surgical treatment would have no effect on survival in patients diagnosed with uterine leimyosarcoma with appendicular bone metastases. METHODS: One hundred fourteen patients diagnosed with uterine leiomyosarcoma and treated at the Department of Oncologic Orthopedics at XXX hospital from 2004 to 2021 met the criteria for inclusion in this retrospective cohort study. The study specifically encompassed patients with histopathologically confirmed appendicular bone metastases secondary to uterine leiomyosarcoma, who underwent either surgical intervention or conservative treatment. Exclusion criteria involved patients with exclusive vertebral bone metastases, as well as those lacking essential examination and follow-up data. Notably, the study included nine follow-up patients with at least 2 years of follow-up who developed appendicular skeletal metastases during the follow-up period. RESULTS: Of the 9 patients, 3 had humeral metastases, 2 had femoral metastases, 1 had femoral and diffuse pelvic metastases, and the other 3 had pelvic metastases. Bone metastases occurred at a mean of 33.3 ± 32.4 months (range 3 - 108) after the diagnosis. After bone metastasis, 6 patients died after an average of 40.3 ± 26.7 months (range 12-84 months). One patient with a pathologic fracture in the proximal humerus underwent resection arthroplasty, 1 patient with metastases in the proximal femur underwent resection arthroplasty, 2 patients with metastases to the femoral shaft underwent curettage-cementation (C&C) and intramedullary nailing, and 1 patient with persistent pelvic pain underwent C&C. No surgery was performed in the other patients. CONCLUSION: In patients diagnosed with uterine leiomyosarcomas, survival did not differ between palliative surgery and conservative treatment after appendicular bone metastases. Patient assessment should be individualized, and overall health should be evaluated before palliative surgery is performed. LEVEL OF EVIDENCE: IV.

Traditional Dual Growing Rod Technique in the Management of Early Onset Scoliosis and Its Effects on Spinal Growth and Lung Development: The Mid-Term Prospective Results.

OBJECTIVE: The purpose of this study was to investigate the safety and effectiveness of the traditional dual growing rod (TDGR) technique, using only pedicle screws for fixation with more frequent lengthening while evaluating scoliosis correction in the growing spine, spinal growth rates, and the differences in lung volumes. PATIENTS AND METHODS: In this single-centre prospective study, 27 patients with a follow-up of over three years were included in the study. Only pedicle screws were used as foundations for fixation. Routine lengthening procedures were performed every six months. Data were recorded including the age of initial surgery, gender, number of lengthenings, follow-up, and complications. The Cobb angle of the major curve, kyphosis angle, T1- S1 length, space available for lung (SAL) ratio, coronal and sagittal balance, and the height of all patients were measured and recorded preoperatively, immediately postoperatively, and finally before and after every lengthening. RESULTS: The average follow-up time was 46.3 months (36-64 months). The correction rate was 69.5% for Cobb angle and 43.2% for kyphosis between preoperative and final follow-up period. The time between two lengthenings was 6.9 months, and the mean T1-S1 length increase was 1.78 cm per year. The SAL ratio increased from 0.885 preinitially to 0.985 at the last follow-up. The complication rate was determined as 9.6% in 187 procedures. Acceptable improvements were determined in the specified parameters with low complication rates with the use of this technique. CONCLUSION: The TDGR technique with proximal and distal pedicle screws as anchors is a safe and effective treatment for deformity control in selected patients with early onset scoliosis (EOS). Repetitive surgical interventions are the negative side of this technique.

Are the women with Sjögren's Syndrome satisfied with their sexual activity? / As mulheres com síndrome de Sjögren estão satisfeitas com sua atividade sexual?

ABSTRACT Objective: Females with Sjögren's Syndrome (SS) often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS. Methods: Forty-six premenopausal women with SS and 47 age-matched controls were studied. Age, duration of the disease, medications, and comorbid diseases were noted. Participants completed 36-Item Short Form Health Survey (SF-36) and Female Sexual Function Index (FSFI). Patients were asked about vaginal discharge and itching in the last month, and if they informed their rheumatologists about any sexual problems. Gynecologic examinations were performed and vaginal smears were taken on each participant. Results: The median total scores of FSFI were significantly lower in the SS group than the controls [17.12 (2.4-27.8) and 27.4 (16.9-36.0), respectively, p < 0.001]. In the SS group, 37 (80.4%) and in the control group 18 (38.3%) of patients were sexually dissatisfied (p < 0.001). Vaginal dryness and lubricant use were significantly increased in patients with SS compared to controls (p < 0.001). Life quality scores were significantly lower in patients with SS than the controls (p < 0.001). Vaginal dryness was negatively correlated with FSFI total (r = −0.312, p = 0.035) and subscores except desire and arousal. Physical functioning, role physical and role emotional scores were positively correlated with total FSFI scores (r = 0.449, p = 0.002, r = 0.371, p = 0.011, r = 0.299, p = 0.043, respectively). Conclusions: Women with SS experience less satisfaction with sexual activity, which can be affected by age, vaginal dryness, physical pain, and impaired function due to the disease. Therefore, rheumatologists should pay attention to these symptoms and management.

RESUMO Objetivo: As mulheres com síndrome de Sjögren (SS) muitas vezes experimentam secura vaginal e dispareunia, juntamente com sintomas glandulares e extraglandulares. Este estudo objetivou avaliar a função sexual e a qualidade de vida de mulheres com SS. Métodos: Estudaram-se 46 mulheres pré-menopáusicas com SS e 47 controles pareados por idade. Avaliaram-se a idade, a duração da doença, os medicamentos usados e as comorbidades. As participantes preencheram o questionário de qualidade de vida 36-Item Short Form Health Survey(SF-36) e o Female Sexual Function Index (FSFI). As pacientes foram perguntadas quanto à presença de corrimento e prurido vaginal no último mês e se haviam informado a seus reumatologistas sobre quaisquer problemas sexuais. Fizeram-se exames ginecológicos e esfregaços vaginais de todas as participantes. Resultados: A mediana do escore total do FSFI foi significativamente menor no grupo SS do que no grupo controle [17,12 (2,4 a 27,8) e 27,4 (16,9 a 36), respectivamente, p < 0,001]. Nos grupos SS e controle, 37 (80,4%) e 18 (38,3%) das pacientes estavam sexualmente insatisfeitas, respectivamente (p < 0,001). A presença de secura vaginal e o uso de lubrificantes foram significativamente mais frequentes em pacientes com SS em relação aos controles (p < 0,001). Os índices de qualidade de vida foram significativamente menores nas pacientes com SS do que nos controles (p < 0,001). A secura vaginal esteve negativamente correlacionada com o FSFI total (r = −0,312 p = 0,035) e com todos os seus subescores, exceto desejo e excitação. Os escores de capacidade funcional, aspecto físico e aspecto emocional se correlacionaram positivamente com a pontuação total do FSFI (r = 0,449, p = 0,002; r = 0,371, p = 0,011; r = 0,299, p = 0,043, respectivamente). Conclusões: As mulheres com SS têm menor satisfação com a atividade sexual, o que pode ser afetado pela idade, secura vaginal, dor física e função prejudicada em razão da doença. Portanto, os reumatologista devem prestar atenção a esses sintomas e seu tratamento.

Efficacy of golimumab on recurrent uveitis in HLA-B27-positive ankylosing spondylitis.

To evaluate the efficacy of golimumab on severe and frequent recurrent anterior uveitis in patients with HLA-B27-positive ankylosing spondylitis. In this study, 15 eyes of 12 HLA-B27-positive AS patients with resistant anterior uveitis who received 50 mg of subcutaneous golimumab (Gol) per month due to frequent uveitis recurrences were analyzed retrospectively between May 2013 and October 2015. Assessment criteria were uveitis activity, the number of recurrence of uveitis, visual acuity, systemic corticosteroid, or other drug requirement for maintenance of remission of AU. Twelve patients (15 eyes) with HLA-B27-positive ankylosing spondylitis and anterior uveitis have been treated with golimumab 50 mg/month. Remission of uveitis was observed in 12 eyes out of 15. Malign hypertension developed in one subject after the second dose of golimumab therefore the treatment was stopped and this subject was excluded from the study. Median follow-up time was 11 months (interquartile range: 8-18). No uveitic reaction was seen except in the patient who stopped treatment. No topical or systemic steroid necessity was needed except in two cases with oral 4 mg systemic maintenance. Visual acuity was significantly increased (p = 0.002). Golimumab may be a new and effective choice for maintaining remission and the prevention of recurrences of severe, resistant anterior uveitis in patients with HLA-B27-positive ankylosing spondylitis.

The efficacy of in situ local autograft in adolescent idiopathic scoliosis surgery: a comparison of three different grafting methods

Background/aim: This study was performed to show the efficacy of in situ local autograft with a comparison of in situ local autograft, local autograft with allograft, and local autograft with posterior iliac crest autograft.Materials and methods: In this prospective randomized study, a total of 65 adolescent idiopathic scoliosis (AIS) patients were separated into 3 groups: Group 1 using local autograft and allograft, Group 2 using local autograft only, and Group 3 using local autograft and posterior iliac crest autograft. Posterior segmental instrumentation was also applied to all patients. The mean follow-up period was 28.5 months (range, 15-40 months). Pseudarthrosis was investigated with the multiplanar and three-dimensional images obtained using multislice computed tomography, thoracolumbar bone single-photon emission computed tomography, and three-phase regional and whole body bone scintigraphy. Results: Pseudarthrosis was not observed in any patient. Fusion was obtained in all patients at the end of the follow-up periods.Conclusion: Similar results were obtained in respect of fusion in all 3 groups. Without the use of additional grafts, sufficient fusion can be achieved with the use of local autograft alone for posterior spinal fusion in patients with AIS.

Handedness and breast cancer characteristics.

PURPOSE: Around 10% of the population is left-handed (LH) and the new data suggests that there is genetic as well as environmental influence on the hand preference. The purpose of this study was to evaluate the relationship between breast cancer and hand preference. METHODS: Breast cancer patients diagnosed and treated at the Department of Medical Oncology, Hacettepe University, Institute of Oncology between March 2006 and May 2010 were included in the study. The preferred handedness was asked in all patients and recorded in the patients' medical database. RESULTS: A total of 898 patients with breast cancer were analyzed for handedness, basic characteristics and survival. The median age was 48 years (range 20-83) and all but one patient were female. Of all, 434 (48.3%) patients were pre, 61 (6.8%) were peri, and 399 patients (44.4%) were postmenopausal. Nearly all of the patients (n=869, 96.7%) were operated and modified radical mastectomy was the most frequent type of surgery (N=654, 72.8%). Invasive ductal carcinoma was observed in 659 (73.3%) and in 659 patients (73.1%)estrogen receptors (ER) were positive. Similarly 569 (63.4 %) patients were progesterone receptor (PR) positive and 181 (20.2%) had HER2 overexpression. Most of the patients had T2 (434; 48.3%), N0 (376; 41.9%) and M0 (830; 92.4%) tumors. Of all, 55 (6.1%) patients were LH and the remaining 843 (93.9%) were right-handed (RH). There was no statistically significant difference between the groups related to ER or PR status, TNM classification, tumor localization, menopausal status, HER2 or histological subtype. However the median age of diagnosis in right-handers was higher (48-46 years, p=0.02). There were 10 (1.1%) patients who had already passed away at the time of analysis. The mean overall survival (OS) for RH patients was 33 months (range 1-281) and for LH patients it was 35 months (range 1-182) (p=0.751). The disease disease-free survival (DFS) was 29 months for both groups (p=1.00). CONCLUSION: In our trial the onset of breast cancer was 2 years earlier in LH patients with similar disease characteristics compared to RH patients. As a consequence, it may be advocated that the screening of LH patients should start earlier.

Effect of port-care frequency on venous port catheter-related complications in cancer patients.

PURPOSE: Subcutaneous central venous port catheters (SCVPC) are of great importance in the treatment of patients with malignancies since they provide secure vascular access. Our aim was to assess the impact of long-term catheter care frequency on the frequency of port-related complications. PATIENTS AND METHODS: Two hundred and seven patients who had not been on active chemotherapy through their SCVPC for at least 3 months were enrolled into the study. Those who received catheter care every 3 months or more frequently were assigned to the frequent care group, and the others to the infrequent care group. The patients were examined for port-related complications and thrombosis including port occlusion. Routinely in our clinic, catheter care was done by using 300 IU of heparin. RESULTS: According to the frequency of SCVPC care, 49 (23.7 %) patients were in the frequent care group and 158 (76.3 %) were in the infrequent care group. Median follow-up of all patients was 671 days (range 133-1712). Median frequency of port care in the frequent care group was 90 days (range 30-90), but 441.5 days in the infrequent care group (range 91-1630). None of the patients experienced port-related severe complications during the follow-up time. None of them presented with port occlusion. When the groups were analysed for thrombus (symptomatic and asymptomatic), there was no statistically significant difference (6.4 vs 13.8 %, p = 0.17). Those patients who had received more than first-line chemotherapy were found to have more thrombi than the patients who were treated with only one type of chemotherapy protocol (28.6 vs 10.2 %, p = 0.01), and the patients who had metastatic disease at the last control were found out to have thrombi more frequently than the non-metastatic patients (24.3 vs 9.3 %) (p = 0.01). CONCLUSIONS: In the present study, there was no difference in port-related severe complications between frequent and infrequent care groups during follow-up. However, the rate of thrombosis was slightly higher in the infrequent port care group.

Inflammatory myopathies: One-center experience.

OBJECTIVE: Our aim was to report our experience with inflammatory myositis. MATERIAL AND METHODS: In total, 60 patients were evaluated retrospectively, and 43 of them (71.7%) were female. The median age was 45 (17-81). Of all patients, 33 (55%) were diagnosed as polymyositis, 22 (36.6%) as dermatomyositis (classical, amyopathic, and malignancy-associated), and 5 (8.33%) as undifferentiated myositis. The 3 patients with malignancy-associated dermatomyositis had lung cancer, nasopharyngeal carcinoma, and endometrial cancer. Two patients with polymyositis had a history of low-grade gastric mixed tumor and thymoma but were diagnosed 7 and 12 years ago, and no recurrences have been reported during the routine controls. RESULTS: All patients, other than two with malignancy-associated dermatomyositis, were treated via immunosuppressive agent, and the third patient with lung cancer was diagnosed later and gave up immunosuppressive therapy. Thirty-nine (65%) of the patients were treated via oral low-dose steroid, and 19 (31.7%) were treated via intravenous high-dose pulse steroid therapy. All patients were treated with steroid, which was tapered by time, and 23 (38.3%) were treated with azathioprine, 6 (10%) were treated with cyclophosphamide, 3 (5%) were treated with methotrexate, and 6 (10%) were treated with isolated steroid therapy at the time of diagnosis. The median follow-up period was 37 (2-135) months. Six patients (10%) have died-3 due to myocardial infarction, 1 due to septic shock, 1 due to malignancy, and 1 with an unknown reason. The 5-year survival rate was 76.9%, and the 10-year survival rate was 40%. CONCLUSION: Other than the high ratio of PM in our series, all other results were compatible with the literature. We faced few resistant diseases; therefore, biologic agents were used rarely.

A confusing case: pulmonary lesions including cavities, isolated left heart endocarditis and inferior vena cava thrombosis in a patient with perforated diverticulitis.

There are numerous causes of pulmonary cavitary lesions as infection (bacterial, parasitic and invasive fungal), Wegener granulomatosis (WG) and other vasculitis, sarcoidosis, malignancy, septic thromboembolism, airways disease (cystic bronchiectasis and bullae), pneumatoceles and traumatic parenchymal laceration. Herein, we present a case with perforated diverticulitis causing pulmonary cavitary lesions and a septic thrombus in the neighboring inferior vena cava.

Does androgen receptor have a prognostic role in patients with estrogen/progesterone-negative and c-erbB-2-positive breast cancer?

Recently, it has been shown that androgen and androgen receptor (AR) also have an important role in the pathogenesis and outcome of breast cancer. However, their significance in different subtypes of breast cancer is still under investigation. The aim of this study was to study the effects of AR on clinicopathological features and prognosis in patients with estrogen and progesterone receptor (ER/PR)-negative, HER2-positive breast cancer. Tumor paraffin-embedded blocks from archives were used for AR study. Data of patients with ER/PR-negative and HER2-positive breast cancer diagnosed at our institute between 1999 and 2010 were recorded and analyzed retrospectively. We studied 36 patients with ER/PR-negative and HER2-positive breast cancer for AR status. Sixteen of them (44.4%) showed AR positivity. The median age was 47 and 56 years for AR-negative and -positive patients, respectively (P = 0.03). The number of postmenopausal patients was higher in the AR-positive than -negative group (56 vs 30%) (P = 0.01). Other demographic data were similar in both group. Histopathological parameters and tumor and nodal stages were similar in both groups. Trastuzumab treatment was more frequently given to AR-positive than -negative patients (94 vs 44%) (P = 0.01). Median follow-up was 47.1 and 34.7 months in AR-negative and -positive groups, respectively (P = 0.03). Relapse occurred in six and four patients in AR-negative and -positive groups. Median progression-free survival (PFS) was similar in both groups (15.7 and 19.6 months in AR-negative and -positive patients, respectively; P = 0.56). Two patients died at 23.4 and 46 months of follow-up in the AR-negative group. There were no deaths in the AR-positive group. Overall survival analyses were not done as a result of an unmet number of events. Median PFS was similar in AR-positive and -negative in that group of patients with ER/PR-negative and HER2-positive breast cancer. However AR-positive patients were more frequently postmenopausal, older, and positive for lymphovascular space invasion. More frequently applied trastuzumab in the AR-positive group might have an effect on the similarity of PFS between the two groups. Studies with higher numbers in this subset of patients with breast cancer will give more robust data.

Nasal Natural Killer/T-cell Lymphoma with Skin, Eye, and Peroneal Nerve Involvement.

UNLABELLED: Nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare disease strongly associated with Epstein-Barr virus and is often localized to the upper aerodigestive tract at presentation. Extranodal NKTL may involve any extranodal site and disease beyond the nasal cavity is highly aggressive, with short survival time and poor response to therapy. Herein we present a 57-year-old male that had been treated with systemic chemotherapy and cranial radiotherapy for nasaltype NKTL in the palate with skin, right eye, and right peroneal nerve involvement. He was given salvage chemotherapy consisting of 3 cycles of ICE and his response to the therapy was satisfactory, except for persistent right drop foot. About 6 weeks later, the patient presented with bilateral total loss of vision and proptosis; therefore, DHAP chemotherapy was started. Unfortunately, after 1 cycle of the second salvage chemotherapy, he died due to severe fungal infection of the hard palate. Despite the fact that involvement of any extranodal site is possible, concurrent involvement of many systems in NKTL patients is unusual. Nasal-type NKTL has a poor prognosis, despite local radiotherapy and systemic chemotherapy. Physicians should be aware of this rare disorder than can only be diagnosed after extensive immunohistochemical studies. CONFLICT OF INTEREST: None declared.

Renal disorder preceding multiple myeloma.

Renal disorders preceding multiple myeloma (MM) is a rare type of presentation for plasma cell dyscrasias. We report a case of 40-year-old female who first admitted to the hospital with symptoms and signs of weight loss, nausea and vomiting, and with findings of proteinuria renal dysfunction and anemia. Renal biopsy revealed light chain deposition and findings mimicking membranoproliferative glomerulonephritis (MPGN). Investigation of the patient for plasma cell dyscrasia was resulted in normal findings. After 28 months, she was re-evaluated and MM was diagnosed.

Gemcitabine and cisplatin in patients with carcinoma of unknown primary site.

The optimal therapy for carcinoma of unknown primary site (CUPS) is still under investigation. In this retrospective trial, we reported the response rates and overall and progression free survival of 23 CUPS patients that were treated with gemcitabine and cisplatin. The mean age of the patients was 54.95 (32-77). Sixteen (69.6%) of them were males and 7(30.4%) females. Totally 109 cycles with a mean of 6 were administered. Thirteen of 23 patients (56.5%) presented with only one metastatic site, and the liver is the most frequent metastatic site (39.1%). Histologic types were adenocarcinoma in 14 patients (60.8%), squamous carcinoma in 1 patient (4.8%), epithelioid cancer in 3 patients (13%) and undifferentiated cancer in 5 patients (21.7%). Three patient achieved a CR (13%), 4 patients achieved a PR (17.4%) and 8 patients had SD (34.8%) with an overall 30.4% response rate. However, 8 patients had progressive disease with a percentage of 34.8%. The median follow-up time was 10 months (3-42 months). The mean and median survival was 12.5 (3-42) months and 10 months (range, 3-42 months) and progression free survival was 5.5 months (range, 0-23 months). Gemcitabine plus cisplatin may be an effective treatment of CUPS. Therefore additional trials are needed especially with new chemotherapeutics.

Unilateral diaphragm paralysis possibly due to cervical spine involvement in multiple myeloma.

OBJECTIVE: To describe a patient with unilateral diaphragmatic paralysis due to multiple myeloma (MM) involving the cervical spine and related structures. CASE PRESENTATION AND INTERVENTION: A 52-year-old female presented with dyspnea, low back and widespread bone pain. She was diagnosed as having MM with vertebral involvement and unilateral paralysis of the diaphragm. She received two cycles of a chemotherapeutic regimen consisting of vincristine-Adriamycin-dexamethasone. The bisphosphonate zoledronic acid was also initiated at the same time. During follow-up, the back pain disappeared and the complaint of dyspnea decreased, although the paralysis persisted. CONCLUSION: As part of the differential diagnosis of bone and back pain, we draw attention to MMand the rare complication of diaphragmatic paralysis due to phrenic nerve involvement.