Assessment of primary radical hysterectomy and neoadjuvant chemotherapy followed by radical hysterectomy in Stage IB2, IIA bulky cervical cancer.

OBJECTIVE: Uncertainty concerning the treatment of Stage IB2-IIA (bulky) cervical cancer is still continuing. In this study, an analysis of Stage IB2-IIA (bulky) cervical cancer was performed. The efficacy of primary radical surgery and neoadjuvant chemotherapy followed by a radical surgery was investigated. MATERIALS AND METHODS: Medical data of 50 patients who were diagnosed with Stage 1B2-IIA (bulky) cervical cancer and treated between 2002-2009 were retrospectively assessed. In the radical surgery group, radical hysterectomy + bilateral pelvic + para-aortic lymphadenectomy were performed. In the neoadjuvant chemotherapy group, a combination of cisplatin/topotecan or paclitaxel/carboplatin was given to the patients and then radical surgery was performed. Each group was evaluated individually. Prognostic factors were determined and survival rates were compared between the groups. Ap value was taken < 0.05 for the statistical significance level for all results. RESULTS: Radical surgery after neoadjuvant chemotherapy was performed in 21 and primary radical surgery in 29 patients. Median follow-up time was 36.0 +/- 14.0 months. Average of the tumor size before treatment was 50.2 +/- 7.6 mm. In the radical surgery after neoadjuvant chemotherapy group, lymphovascular space invasion (LVSI) and tumor size (before and after treatment) were determined to be significant factors for each of disease-free survival (DFS) and overall survival (OS). On multivariate analysis, tumor size (before treatment) was found to be an independent prognostic factor for both of DFS (p = 0.006) and OS (p = 0.010). No significant difference in survival periods was observed among the groups. CONCLUSION: There was no significant superiority among the two treatment options. Nonetheless, further studies are needed to compare the multimodal approaches in these stages of cervical cancer.

Clinicopathological features and prognostic factors of the uterine sarcomas: 20 years of experience at Cukurova University.

OBJECTIVE: Uterine sarcomas (US) are rare, malignant, and aggressive tumors of the uterus. In this study the authors aimed to evalu- ate retrospectively the clinical and pathologic features and to investigate the prognostic factors of the U.S. patients who were treated in their department in the last 20 years. MATERIALS AND METHODS: The archive files, medical, and pathological records of the 132 US patients who were operated on and regularly followed up in the clinic between March 1991-March 2011 were reviewed. Clinical features, operation characteristics, pathological findings, adjuvant therapies, and follow-up data of the patients and their effects on survival were investigated. Analysis of disease-free survival (DFS) and overall survival (OS) were calculated using Kaplan-Meier and Cox regression tests. Thep value was taken <0.05 to maintain the statistical significance level for all results. Results: Seventy of the patients were diagnosed with leiomyosarcomas (LMS), 33 were with carcinosarcomas, 12 were with endometrial stromal sarcomas (ESS), nine were with undifferentiated endometrial sarcomas, five were with adenosarcomas, and three were with botryoid rhabdomyosarcomas. The average patients' age was 53.7 +/- 12.6 (17-78). About two-thirds of the patients were in postmenopausal and one-third were in pre- menopausal period. Vaginal bleeding was detected as the most common reason for patients' admission (68.9%). All cases underwent surgery and a procedure of total abdominal hysterectomy + bilateral salpingo-oophorectomy (TAH + BSO) was performed for most of them (88%). The mean duration of follow-up was 36 months (4-198). The two- and five-year OS rates were 65% and 36%, respectively, with a median time of 37 months (95% CI, 28-45). The two- and five-year DFS rates were 59% and 33%, respectively, with a median time of 29 months (95% CI, 18-40). CONCLUSION: As a result of multivariate analysis, while age, stage, lymphovascular space invasion (LVSI), and lymphadenectomy were found to be independent prognostic factors affecting DFS, only stage was detected as an independent prognostic factor for OS.

Hermansky Pudlak Syndrome and Pulmonary Alveolar Proteinosis at the same patient: first case report in the world literature.

Hermansky-Pudlak Syndrome (HPS) is a rare autosomal recessive disorder presenting with oculocutaneous albinism, bleeding diathesis and lysosomal accumulation of ceroid lipofuscin which leads to interstitial fibrosis in lung. Pulmonary fibrosis which is usually associated with HPS-1 and HPS-4 subtypes usually manifests in the third/fourth decades of life representing with giant lamellar bodies of alveolar type-II-cells and their apparent degeneration causes restrictive lung disease. Pulmonary manifestation of this syndrome may lead to premature death. Pulmonary Alveolar Proteinosis(PAP) is another rare disease characterized by alveolar deposition of surfactant phospholipids and proteins secondary to defective clearance by alveolar macrophages. PAP may occur as autoimmune diseases and/or secondary to toxic inhalation, systemic infections or hematological disorders. None of the cases were reported secondary to HPS according to the best our knowledge. As well, pulmonary involvement of HPS was never reported as PAP. We report the first case of PAP in a patient with HPS.

Inheritance of a Chromosome 3 and 21 Translocation in the Fetuses, with One also Having Trisomy 21, in Three Pregnancies in One Family.

The majority of chromosome rearrangements are balanced reciprocal and Robertsonian translocations. It is now known that such abnormalities cause no phenotypic effect on the carrier but lead to increased risk of producing unbalanced gametes. Here, we report the inheritance of a translocation between chromosomes 3 and 21 in a family with one of two fetuses with Down Syndrome carrying the same translocation and the other also carrying the same translocation without the additional chromosome 21. Chromosomal analysis from fetal amniotic fluid and peripheral blood lymphocytes from the family were performed at the Çukurova University Hospital at Adana, Turkey. We assessed a family in which the translocation between chromosomes 3 and 21 segregates: one of the three progenies carried the 47,XX,+21,t(3;21)(q21;q22) karyotype and presented with Down Syndrome; another of the three progenies carried the 46,XX,t(3;21) (q21;q22) karyotype and the third had the 46,XY karyotype. Their mother is phenotypically normal. Apparently this rearrangement occurred due to an unbalanced chromosome segregation of the mother [t(3;21)(q21;q22)mat]. This family will enable us to explain the behavior of segregation patterns and the mechanism for each type of translocation from carrier to carrier and their effects on reproduction and numerical aberrations. These findings can be used in clinical genetics and may be used as an effective tool for reproductive guidance and genetic counseling.

Preoperative evaluation, clinical characteristics, and prognostic factors of nongenital metastatic ovarian tumors: review of 48 patients.

OBJECTIVE: To evaluate the clinicopathologic characteristics, methods for preoperative evaluation, prognostic factors, and overall survival of nongenital ovarian metastases (NGOM). MATERIAL AND METHODS: Forty-eight patients with NGOM followed between January 2001 and January 2009 in Cukurova University Department of Gynecologic Oncology were included in the study. Clinical characteristics including demographics, preoperative imaging methods, endoscopic evaluations, tumor markers, histopathologic findings, prognostic factors, types of surgery, modalities for adjuvant therapy and survival were analyzed. RESULTS: The gastrointestinal tract is the most common location of the primary tumor; colonic origin was found in 41% of the patients (n = 20). All metastatic lesions were adenocarcinoma with 23% of these classified as Krukenberg and 29% as mucinous type adenocarcinoma. When the whole group was evaluated, median survival time was 15.7 months in patients and there were significant differences between the groups according to primary site. Histopathological subtypes and presence of peritoneal carcinomatosis affected the median survival. The significant prognostic factors were primary site and histopathologic subtypes of the NGOM. CONCLUSIONS: NGOM should be kept in mind to avoid inappropriate management and therapy in patients with surgically managed ovarian tumor, especially young patients with gastrointestinal complaints.

Prognostic factors determining recurrence in early-stage endometrial cancer.

OBJECTIVE: This study aimed to determine the clinically important prognostic factors for loco-regional or distant recurrence in early-stage endometrial cancer. MATERIALS AND METHODS: This study complied with the Declaration of Helsinki, and the local ethics committee approved the study. Cases who underwent primary surgery of early-stage endometrial cancer at the Institution from 2000 to 2012 were reviewed retrospectively. Patients who did not detect recurrence were classified as group 1 (n = 200); those who detected recurrence were classified as group 2 (n = 23). Clinically prognostic factors were evaluated by univariate analyses. RESULTS: The average age for group 2 (LUSI) was 63.8 years (p = 0.0001). Patients with grade 3 histology were all detected within group 2 (p = 0.0001). Endometrioid adenocarcinoma displaying squamous differentiation was found with a rate of 58.3% in group 2 (p = 0.0001). Lower uterine segment involvement (LUSI) and lymphovascular space invasion (LVSI) rates were 86.9% in group 2 (p = 0.0001). The rate of tumor size > 2 cm was 56.6% in group 2 (p = 0.0001). The median depth of myometrial invasion (DMI) was 5.1 mm (p = 0.034) and the average in myometrial thickness was 14.5 mm in group II (p = 0.0001). The percentage of myometrial invasion was 35.8% in Group II (p = 0.0001). Tumor free-distance was 9.4 mm in group II (p = 0.0001). CONCLUSION: Age and clinicopathological parameters of the tumours are significant predictors for recurrence in early-stage endometrial cancer.

Mullerian adenosarcoma of the uterine cervix in an adolescent girl.

BACKGROUND: Cervical adenosarcoma is a rare gynecological tumor which is prone to recur locally. A case of cervical adenosarcoma in a 14-year-old girl treated in order to preserve fertility. CASE: A 14-year-old girl presented with a pedunculated mass protruding from the vagina. Simple excision was performed. Histological evaluation revealed adenosarcoma. Her clinical course has been uneventful for fifteen months. SUMMARY AND CONCLUSION: Adenosarcoma is a low malignant potential tumor of the female genital tract. A simple excision may be an option for young women who need to preserve their fertility. It is mandatory to monitor regularly the cervical base of the lesion for the purpose of the high potential of the local recurrence.

Coexistence of cystic intra-abdominal lymphangiomas and diffuse venous haemangiomas in adult life.

Diffuse haemangioma and intra-abdominal lymphangioma are rare in adults. In this case report, we present a 33-year-old female with coexisting multiple cutaneous and visceral cavernous haemangiomas and two huge intra-abdominal lymphangiomas of 25 and 35 cm in diameter. The organs involved were the liver, pericardium, renal hilus and bladder. She died due to disseminated intravascular coagulation and multiorgan failure, which resembled Kasabach-Merritt syndrome. The coexistence of generalised haemangiomas and intra-abdominal lymphangiomas and the lack of complaints until the age of 33 years makes her an unusual case in the literature. We also emphasise the other clinical conditions that should be considered in the differential diagnosis.

A case of bacillary angiomatosis presenting as leg ulcers.

We report a 32-year-old immunocompetent man who had multiple leg ulcers caused by bacillary angiomatosis without a history of direct contact with cats. Bacillary angiomatosis should be kept in mind in the differential diagnosis of leg ulcers in cases of unknown aetiology.

Vasculitis induced by zafirlukast therapy.

Zafirlukast is a leukotriene inhibitor that has recently been approved for the prophylaxis of asthma. Although this new product has been well accepted because of its convenient dosing and relatively few side effects, several cases of Churg-Strauss syndrome have been reported to be associated with its use. In this paper we describe the case of a 54-year-old white man with no history of corticosteroid therapy in whom leukocytoclastic vasculitis, hepatitis and eosinophilia developed while he was on zafirlukast therapy for mild asthma.

Multiple myeloma with skin involvement.

Multiple myeloma (MM) is a plasma cell dyscrasia seen in the elderly that constitutes 10% of all haematopoietic neoplasias. It is a systemic disorder affecting various organs, in particular the kidneys and bones. Skin involvement is not a common finding. This report presents a case of MM with skin involvement.

Environmental malignant mesothelioma in southern Anatolia: a study of fifty cases.

Malignant mesothelioma is a highly aggressive tumor of the serous membranes, which in humans results from exposure to asbestos and asbestiform fibers. Although occupational malignant mesothelioma is still the most common form of this lesion, naturally contaminated soil can play an important role in the development of environmental malignant mesothelioma in some parts of the world. Fifty cases of malignant mesothelioma (MM) from southern Turkey with no occupational history of asbestos exposure were reviewed regarding pathologic and clinical features. A case of hyaline fibrous plaque of the pleura was also included in this series. Histologically the cases were classified as epithelial (36 cases); sarcomatous (7 cases); and biphasic (7 cases). One of the sarcomatous cases was desmoplastic. Ultrastructural examination of the tumor tissue in three cases revealed long-surface microvilli in epithelial cells. Interstitial cells of the lung in one case showed electron-dense asbestos fibers in the cytoplasm. Mineralogical analyses of the lung tissue in three cases of MM and the case of pleural plaque showed high amounts of asbestos fibers most consistent with tremolite and actinolite. The clinical and pathologic features of our cases support that the environmental inhalation of asbestos is still a major health problem in some parts of Turkey.

Callotasis in nonvascularized periosteal bone grafts and the role of periosteum: a new contribution to the concept of distraction osteogenesis.

Bone lengthening by slow, progressive distraction has gained widespread acceptance. In this study we investigated the possibility of distraction osteogenesis in nonvascularized periosteal bone grafts, and assessed callus formation and callotasis by means of radiological and histological examination with the aim of determining the role of the periosteum. The process of distraction osteogenesis in nonvascularized bone grafts was studied histologically and radiologically in 22 growing rabbits. The metatarsal bone grafts taken from the rabbits were divided into two groups. Group 1 contained 15 bone grafts covered with periosteum and group 2 (the control group) contained 7 bone grafts without periosteum. These grafts were subjected to osteotomy and then placed in the lumbar pocket. After 10 days, distraction was started and continued at 0.5 mm per day for 10 days. Thus an elongation of 4 to 6 mm was achieved in both groups. Radiological examination was performed postoperatively and after 10, 15, 20, and 30 days of starting the distraction. Histological examination was performed after 15, 20, and 30 days. Radiologically, progressive calcification and, histologically, both intramembranous and endochondral ossification were detected in group 1. However, in the control group (group 2), bone lengthening failed. Our study demonstrated the possibility of distraction osteogenesis in periosteal bone grafts, and provides information regarding the importance of periosteum as well as its osteogenic capacity.

Fine needle aspiration cytology of hepatoblastoma. A report of two cases.

BACKGROUND: Hepatoblastoma is the most common primary hepatic tumor in children. The literature contains few examples of fine needle aspiration (FNA) cytology of these tumors. CASES: A 5-month-old and 4-month-old underwent ultrasonography-guided FNA for the preoperative investigation of hepatic masses. The smears were stained with May-Grünwald-Giemsa and Papanicolaou stain. Alcohol-fixed smears were used for immunocytochemistry. All smears revealed cells with round/oval nuclei, prominent nucleoli and vacuolated cytoplasm, arranged in groups and acinar structures. The groups were embedded in a myxoid stroma. alpha-Fetoprotein was positive in all, and vimentin was positive in some tumor cells. The cytologic findings resembled the histologic counterpart in one case, and the other case agreed with the clinical/radiologic prediagnosis. Immunocytochemistry was supportive. CONCLUSION: FNA cytology can be diagnostic in many other childhood tumors as well as hepatoblastomas. Detailed descriptions of cytomorphologic features of hepatoblastoma will help FNA to be used confidently on these tumors.

Histologic alterations in the thyroid gland after fine-needle aspiration.

To evaluate the histologic alterations due to the fine-needle aspiration (FNA), a comparative study between 20 aspirated and 20 nonaspirated thyroidectomy specimens was performed. The most common findings in the aspirated group were hemorrhage (80%) and vascular proliferation and/or vascular thrombosis (45%). In one of the aspirated cases with the cytologic diagnosis of follicular neoplasm, histologic sections revealed prominent vascular and endothelial proliferation. Fibrosis, cystic degeneration, and infarction were other histologic findings in the aspirated group. Hemorrhage was seen in 45% and cystic degeneration in 25% of the nonaspirated cases. Fifty percent of the nonaspirated cases did not have any additional findings. In conclusion, knowledge of previous FNA application and awareness of possible histologic alterations due to the needling is necessary while evaluating the histologic sections of the thyroidectomy specimens.