Survival outcomes and prognostic factors of endometrial stromal sarcoma and undifferentiated uterine sarcoma.

PURPOSE: To review the diagnostic and therapeutic procedures of patients diagnosed with Endometrial Stromal Sarcoma (ESS) and Undifferentiated Uterine Sarcoma (USS) at our institution and investigate their clinical outcomes and factors affecting prognosis. METHODS: We retrospectively collected demographic data, preoperative diagnostic methods and therapeutic management of patients treated for ESS and UUS between January 1995 and December 2019 at Vall d'Hebron Barcelona Hospital Campus, Spain. Overall survival and disease-free survival were calculated. Cox proportional-hazards regression models were calculated. RESULTS: Sixty-three patients were included in the study, of which 51(81%) had a diagnosis of ESS and 12(19%) of UUS. Twenty patients (31.7%) were diagnosed after a previous non-oncologic surgery, and 12 of them (60%) suffered from tumor disruption. Cytoreductive procedures were needed in 29 patients (46%), and optimal cytoreduction was achieved in 80.9% of the patients. The median follow-up was 7.6 years (IQR = 0.99-14.31). Five-year overall survival was 57.6% (44.2-68.8) and was significantly better for low-grade ESS (LG-ESS) patients (p < 0.01). Five-year disease-free survival was 57.1% (42.8-69.1) and was also significantly higher in LG-ESS cohort (p = 0.03). After multivariate analysis histological type, age, FIGO stage, optimal surgery and mitotic index were found significantly correlated with survival. For high-grade EES (HG-ESS) and USS patients adjuvant radiotherapy also correlated with improved survival. CONCLUSION: Overall survival and disease-free survival are significantly better in patients with LG-ESS cohort. HG-ESS and UUS show similar survival outcomes. Age, FIGO stage, optimal surgery and histological type were significantly correlated with survival in the global cohort, whilst adjuvant radiotherapy correlated with improved survival in HG-ESS and UUS patients.

Analysis of results of soft tissue sarcoma margins revision surgery. / Análisis de resultados en cirugía de revisión de márgenes de sarcomas de partes blandas.

INTRODUCTION: Patients from other centres where they have had an unplanned surgical resection of a soft tissue sarcoma are often referred to hospitals specialised in sarcomas. MATERIAL AND METHODS: A study was conducted on 35 patients who required this type of surgery were referred to our center between November 2001 and July 2013. RESULTS: Surgery had been performed on 29% of the patients without any complementary tests being done. In 75% of cases, the sarcoma diagnosis was discovered in the post-surgical histological study. Synovial sarcoma was the most common, affecting 38% of the patients. A surgical revision of the margins was performed on all of them, and adjuvant treatment was performed on 86% of them. The histopathology study found that 69% of the patients had residual disease. At the end of follow-up, 12% had a local recurrence, another 12% distant metastases, and 3% had died. CONCLUSION: Given the results, it is concluded that any tumour of the soft tissues in which malignancy is suspected has to be resected in a reference centre. If an unplanned esection was performed in another centre, it should be referred immediately in order to perform an imaging study, revision surgery, and if required, adjuvant treatment.

[Cholestasis and listeriosis in the third trimester of pregnancy]. / Colestasis y listeriosis en el tercer trimestre de la gestación.

Listeriosis is an infection produced by Listeria monocytogenes. It is infrequent and affects people at extreme ages, pregnant women, immunocompromised people and, occasionally, healthy people. Its incidence has increased in recent years and shows a certain tendency to seasonality, increasing in summer. It can appear sporadically or as outbreaks. In pregnant women the infection is most frequently produced in the third trimester and the symptoms are usually light. Nonetheless, the infection of the fetus is severe, and can produce miscarriages, fetal deaths, corioamnionitis and premature births with the newborn infected, manifested in the form of granulomatosis infantiseptica with abscesses and scattered granulomas or at a later stage , as meningitis or sepsis. Intrahepatic cholestasis is a reversible form of cholestasis, its cause is unknown, it is specific to pregnancy and is more frequent in multiparous women, in the third trimester and rarely before the 26th week. It disappears following childbirth and is the second cause of jaundice in pregnancy, after hepatitis. The diagnosis of cholestasis is basically clinical. It appears as palmoplantar pruritus but can also produce nausea, vomiting and abdominal discomfort localized in the right hypochondrium. Given that listeriosis and cholestasis can have a shared symptomology, the possibility of listeriosis must be borne in mind in order for early implementation of the mechanisms of diagnostic confirmation (cultivation of sterile fluids or tissues: blood, neonatal CSF, amniotic liquid or placenta) and specific treatment. We present a case of cholestasis and listeriosis in the third trimester with a good maternofetal result.

Sentinel lymph node identification in a primary ductal carcinoma arising in the vulva.

Primary or metastatic breast-like carcinoma of the vulva is a rare event. Because of the similarity with breast ductal carcinoma, we think that the same principles used for treatment of orthotopic breast cancer can be applied, as well as the use of sentinel lymph node technique, which is widely accepted in the management of early-stage breast cancer. We report a 49-old-year postmenopausal woman who was referred to our institution after small biopsy of a 3.5- x 3-cm right vulvar tumor. Histopathologically, infiltration of the vulvar dermis by a ductal carcinoma of mammary gland type was reported. At operation, the sentinel node technique revealed two sentinel nodes in the right inguinal area. Although these nodes proved negative for malignancy, the patient underwent wide local excision of tumor and complete ipsilateral inguinofemoral lymphadenectomy. The remaining excised nodes were negative. Surgical specimen proved estrogen- and progesterone-positive receptors, the reason for which the patient received tamoxifen adjuvant therapy. This report represents the first case in the world literature of primary breast carcinoma arising in the vulva in which sentinel lymph node identification has been possible. Because of the rarity of this condition, the pathologic similarity of this tumor along with currently accepted guidelines for the management of breast cancer supports the possibility of local excision and sentinel lymph node identification as a possible alternative to inguinofemoral lymphadenectomy.

Carcinoid tumor of the uterine corpus. A case report.

BACKGROUND: Carcinoid tumors are neoplasms of neuroendocrine origin that rarely affect the genital tract. CASE: A 75-year-old woman underwent hysterectomy and bilateral adnexectomy due to vaginal bleeding and uterine pathology (leiomyoma, cervical low grade squamous intraepithelial lesions and endometrial hyperplasia on ultrasound). Pathologic examination of the specimen disclosed a uterine corpus carcinoid tumor. The patient had been taking tamoxifen for adjuvant treatment of breast cancer diagnosed and treated seven years before. CONCLUSION: A review of the literature revealed one case of carcinoid tumor of the uterine wall. There does not appear to be any relationship between tamoxifen and the carcinoid tumors reported.

Merkel cell carcinoma of the vulva.

Merkel cell carcinoma is a very rare tumor. This is why it is not known whether this neoplasm behaves differently in the vulvar location than at other sites. We present a patient with a Merkel cell carcinoma assessed with a light optical microscope, immunohistochemistry, and electron microscope. Only eight previous cases have been reported in the literature. We discuss pathologic findings, such as histologic trabecular pattern under the optical microscope and neurosecretory granules (similar to Merkel cell carcinoma of the skin) under the electron microscope. Also discussed are the results of immunohistochemistry for low-molecular-weight cytokeratin, neuron-specific enolase, chromogranin, and Leu 7, and molecular study of N-ras, K-ras, N-myc, and p53 genes. Little is known about Merkel cell carcinoma of the vulva, but it seems to have a more aggressive behavior and poorer prognosis than Merkel cell carcinoma at other sites.

Malignant vulvo-vaginal melanoma: a report of 7 cases.

To evaluate the clinical outcome of patients suffering from primary vulvo-vaginal malignant melanoma retrospective review of clinical and pathological data of six patients with vulvar malignant melanoma and one patient with vaginal melanoma was done. Patients were staged using the 1988 FIGO histological classification for vulvar cancer, and the 1992 American Joint Committee on Cancer (AJCC) classification for malignant melanoma of the skin. Clinical features, type of surgery, adjuvant therapy, recurrences, distant metastasis, and survival were recorded. Of 174 primary cancers of the vulva, six were malignant melanomas (3.4%), the second most common cause of vulvar cancer. Within the same period, only one primary vaginal melanoma was detected among 57 primary vaginal cancers (1.7%). The median age of all patients was 71 (range 61-84). We performed different surgical procedures, including a radical local excision of the lesion in the vaginal melanoma, a radical hemivulvectomy in one case, a radical vulvectomy in an other case, and a radical vulvectomy with lymph node dissection en bloc in three cases. According to the 1988 FIGO classification for vulvar carcinomas, three patients were diagnosed as stage I and four as stage III melanomas; and using the 1992 AJCC classification for malignant melanoma of the skin, all patients were staged as stage III. Two out of five patients, with a follow-up longer than two years, continued to live and were disease free at the last check-up (40%): the rest of them (60%) died of disease 2.9 months after the diagnosis of the distant metastasis. Vulvo-vaginal malignant melanomas are neoplasias with a very poor prognosis and a short period of time between the detection of distant metastasis and death. Although treatment is not clear in vaginal melanoma, the most accepted treatment in vulvar melanomas is the radical local excision of the lesion with a homolateral inguinofemoral lymphadenectomy.

Uterine sarcoma: a clinicopathological study of 93 cases.

OBJECTIVE: To evaluate the clinical outcome of patients suffering from primary uterine sarcoma diagnosed and treated in our Hospital. SETTING: Department of Gynecologic Surgery/Gynecologic Oncology, Hospital Universitario Materno-Infantil Vall d'Hebron de Barcelona, Barcelona, Spain. SUBJECTS AND METHODS: A retrospective review from 1967 to 1995 of clinical and pathological characteristics of 93 patients with primary uterine sarcoma was done. Patients were staged using the 1988 FIGO histological classification for uterine cancer. Clinical features, type of surgery, adjuvant therapy, recurrences, distant metastasis, and survival were recorded. RESULTS: Our study included three main histologic types: 44 patients with leiomyosarcoma, 26 patients with endometrial stromal sarcoma, and 18 patients with mixed Müllerian sarcomas. The mean age for all patients was 54.8 years, and the most common symptom was vaginal bleeding. Other clinicopathological features were examined. Although surgery was the most frequent treatment, adjuvant therapies have been analyzed and discussed. The overall three-year survival rate was 67.9% and the overall five-year survival rate was 64.5%. We found statistical differences (p < 0.001) between the stage I survival rate and other stage survival rates. CONCLUSIONS: Uterine sarcoma is an uncommon neoplasia diagnosed in the 6th decade of life. Leiomyosarcoma is the most frequent histologic type (47.3%). Stage I uterine sarcoma has a better prognosis than other stages.

Cardiac tamponade by aortic dissection in a hospital without cardiothoracic surgery.

A 48-year-old male patient had arrest due to rupture of a dissecting aneurysm of the thoracic aorta into the pericardial cavity with tamponade; he received treatment in a general hospital lacking the facilities to practice heart surgery. The patient was treated by means of intermittent pericardial drainage while being transferred to another hospital at 100-km distance for surgical treatment.

[Pilomatrixoma. Review of 179 cases]. / Pilomatrixoma. Revisión de 179 casos.

We introduce a study about 179 cases of Pilomatrixioma, registered in the archives from General Hospital "Vall d'Hebrón" of Barcelona between 1972-1988. Tumour of pediatric presentation, about 502 of cases have been observed in young people under 20 years, being both sexes almost equally affected percentually. About 75% of cases appear a a a single tumour smaller than 15 mm of diameter. Multiple presentation is observed only in the 2.8 of cases. A bit more than the 50% of cases are located in the head and the neck, usually in the preauricular region. Next follow upper extremities (27%), lower extremities (11%), and the rest of the body (6.7%). Recidivation appear only in the 0.55% of cases.