Sarcomas en la extremidad superior: revisión del Servicio de Cirugía Plástica - Comité de Sarcomas de Partes Blandas del Hospital Dr Negrin / Upper extremity sarcomas. Review from Plastic Surgerym Department - Soft-Tissue Sarcoma Committee at Dr. Negrin Hospital

Introducción y Objetivo. Los sarcomas de la extremidad superior suponen un bajo porcentaje dentro de los sarcomas en general. Las características anatómicas de las extremidades superiores representan un reto quirúrgico a la hora de conjugar la radicalidad con la preservación funcional de la extremidad. Por el contrario, la raíz de la extremidad ya presenta otras características anatómicas y dimensiones, permitiendo extirpaciones más amplias y siendo además asiento favorito de sarcomas de bajo grado. Con estas premisas, es de suponer que el pronóstico global de esta localización sea más favorable que el del resto de la extremidad. El objetivo del presente estudio es confirmar dicha hipótesis en base a nuestra propia experiencia. Material y Método. De los 131 sarcomas intervenidos en nuestro Servicio durante los últimos 10 años, recogemos 29 casos de sarcomas en extremidad superior, que dividimos en 2 grupos según su asiento: los que afectan a la cintura escapular (hombro, escápula y clavícula), con 12 casos; y los de asiento en la extremidad propiamente dicha (axila, brazo, antebrazo y mano) con 17 casos. Analizamos una serie de variables en ambos grupos: sexo, edad, localización, tipo histológico, grado de diferenciación, presentación, tipo de extirpación, tratamiento quirúrgico, terapias complementarias, estado local, estado general y seguimiento. Resultados. Los sarcomas de la cintura escapular fueron, en nuestro grupo de estudio, de menor grado que los de la extremidad propiamente dicha. Se presentaron en su mayoría como casos primarios. Estos sarcomas son susceptibles de tratamientos más radicales y como consecuencia, los márgenes obtenidos fueron mejores que los del otro grupo. Ninguno de los 12 pacientes con sarcoma en cintura escapular presentó enfermedad local o general. Los sarcomas de la extremidad superior propiamente dicha fueron de predomino femenino, de más alto grado y se presentaron como recidivas o persistencias en más de la mitad de los casos. Las posibilidades quirúrgicas locorregionales son menores. Se practicaron 3 amputaciones y 1 desarticulación. Se emplearon colgajos libres en 5 pacientes, 2 de ellos con componente óseo. El 47% de los pacientes recibió alguna forma de radioterapia y 2 pacientes recibieron quimioterapia. Contabilizamos 4 pacientes fallecidos, lo que supone un 24% del total de los casos de nuestro grupo. Conclusiones. Las características en nuestra serie de sarcomas de la raíz de la extremidad y de la extremidad propiamente dicha son distintas. En la primera las presentaciones primarias fueron más frecuentes, los grados de desdiferenciación más bajos y las extirpaciones más amplias, lo que se traduce en menor enfermedad local, general y mortalidad. Por el contrario, las presentaciones iniciales de los sarcomas de la extremidad (brazo, antebrazo y mano) fueron con frecuencia tras resecciones no planeadas, lo que se traduce en persistencias o recurrencias. Además, el grado de desdiferenciación de los sarcomas en esta zona es más alto y las extirpaciones que se consiguen no son tan amplias, por lo que los resultados en cuanto a enfermedad local, general o muerte por la enfermedad son peores (AU)

Background and Objective. Upper extremity sarcomas represent just a small percentage of all sarcomas. The anatomic characteristics of upper extremity challenge the goal of combining radicality and limb functional preservation. On the other hand, the limb root presents other features regarding dimensions and anatomy thus allowing wider resections. Moreover it is a common place for low grade sarcomas. With such premises it can be considered that the prognosis of sarcomas of this location is more favorable than those of the rest of the extremity. The aim of this study is to confirm this hypothesis, based on our own experience. Methods. During the last 10 years 131 sarcomas were operated in our Unit, 29 of them affecting the upper extremity were recruited. They were further subdivided into 2 groups according to their location: shoulder girdle (shoulder, scapula and clavicle) with 12 cases, and those arising at the limb itself (axilla, arm, forearm and hand) with 17 cases. Several variables were analyzed in both groups: gender, age, location, hystologic type, differentiation grade, presentation, extirpation regarding margins, surgical treatment, complementary therapies, local status, general status and follow - up. Results. Sarcomas of the shoulder girdle own lower grade than those of the extremity itself. They present mostly as primary cases.Wide resections were feasible and therefore the obtained margins were better than those of the other group. None of the 12 patients of this group has had neither local or general disease. Sarcomas of the extremity itself appeared more in women, with higher grades and they are seen firstly at our institution after inadequate surgical margins or as recurrences in more than half of the cases. Locoregional flap options are scarce. Three amputations and 1 shoulder disarticulation were done in this group. Five free flaps, 2 of them with osseous component, were used. Forty-seven patients received some kind of radiotherapy and 2 more patients received chemotherapy. Four deads were recorded among this group representing 24% of all cases. Conclusions. In our series the characteristics of sarcoma to the shoulder girdle and those of the extremity itself were different. In the former primary presentations as new cases are more frequent, dedifferentiation grades were lower and resections wider resulting in lower rates of local recurrences, general disease or mortality. On the other hand, sarcomas to the extremity appear mainly after unplanned resections as recurrences or inadequate margins excisions. Dedifferentiation grades here are higher and resections are not as wide as in the other group. Worse results regarding local disease, general disease or mortality were here recorded (AU)

Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma.

BACKGROUND: Current National Comprehensive Cancer Network guidelines for the treatment of retroperitoneal sarcomas (RPS) endorse surgical resection, but the role of radiotherapy (RT) is less clear. We investigate the utilization and benefits of intraoperative RT (IORT) in the treatment of RPS. METHODS: We queried the Surveillance, Epidemiology and End Results (SEER) database (1988-2013) for the utilization of IORT and perioperative external beam RT (EBRT) in patients who underwent surgical resection of RPS. Groups were defined as any IORT (aIORT), IORT alone (IORT-), IORT with EBRT (IORT+) and preoperative and/or postoperative EBRT without IORT (EBRT). Demographics, tumor characteristics, extent of disease, and survival were compared between groups. RESULTS: We identified 908 patients with RPS who underwent surgical resection with perioperative RT. Demographics of age, sex, and race were similar between groups. There was no difference in baseline tumor characteristics of mean size, tumor grade, or histological subtype between groups. A higher percentage of patients receiving aIORT had tumors >20 cm in size, and extension beyond local tissues. Liposarcoma and leiomyosarcoma were the most common subtypes overall and in each subgroup. Patients with liposarcoma undergoing IORT and EBRT (IORT+) demonstrated a survival benefit over both IORT alone (IORT-) and EBRT alone. CONCLUSION: IORT was used infrequently for RPS but generated equivalent outcomes compared to EBRT, despite being utilized more often for larger tumors and those with peri-tumoral soft-tissue invasion. Patients with the most common subtype (liposarcoma) may benefit from combination IORT with adjuvant EBRT versus other regimens.

Well-differentiated liposarcoma of the epiglottis.

OBJECTIVE: Liposarcomas of the larynx and its sub-sites are rare. Within this group of tumours, well-differentiated liposarcomas of the epiglottis have been only sporadically reported. In view of its infrequent presentation, difficulty still exists over the diagnosis of this pathological entity, together with uncertainty over its exact accepted management. METHOD: We present the case of a well-differentiated liposarcoma of the epiglottis, and we review the associated English-language literature. RESULTS: The reported patient underwent multiple attempts at surgical treatment prior to establishment of a firm diagnosis. In light of the diagnosis and other mitigating factors, a conservative approach was adopted. CONCLUSION: A high index of clinical suspicion and detailed histological analysis are required when encountering a recurrent soft tissue lesion of the larynx. In the presented case, a multidisciplinary approach and conservative management plan were adopted, based on a holistic management approach and a review of the published literature.

Clinical considerations on the retroperitoneal liposarcomas.

AIM: It presents a clinical case of undifferentiated retroperitoneal liposarcoma with 5 years' recurrence from the first operation for the rarity of the occurrence, the problems related to surgery and complementary therapeutic approach. MATERIAL OF STUDY: Male patient aged 73 was operated for removal of retroperitoneal mass with involvement of the right kidney at the Second University of Naples in the 2003. In accordance with the interdisciplinary board, complementary therapy is not indicated and follow-up program. The successive controls were negative until at least 2007. Reoperation for recurrence in 2008 for the presence of massive bone formation occupying a large part in the right half of the abdomen at the sub-hepatic level. In both cases histological examination showed undifferentiated liposarcoma. DISCUSSION: It is of unknown etiology and only 25% occurs in well-differentiated cells are also more than 100 histological subtypes, 85% are malignant. The most affected is the male sex and from the beginning looks like malignancy. The trend of growth in general is slow, and in most cases tends to recur over time. The role of chemotherapy and radiation therapy is controversial. CONCLUSIONS: The peculiarity of our case is higher than the average survival despite advanced age and presence of recurrence. This confirms the importance of surgical treatment, thus offering the patient a chance of better long-term survival.

Liposarcoma of the head and neck: The University of Texas M. D. Anderson Cancer Center experience.

BACKGROUND: Our aim was to review our experience with liposarcoma of the head and neck region. METHODS: This is a retrospective case series at a comprehensive cancer center (1945-2005). RESULTS: Of 30 patients, 10 (33%) were initially misdiagnosed. Local recurrences were common (overall rate = 53%), and 4 patients (13%) developed distant metastases. Decreased crude disease-specific survival rates were significantly associated with recurrence (especially distant recurrence [0%]), age less than 38 years (40%), and pleomorphic subtype (45%); however, in Kaplan-Meier analyses, only larger tumor size, negative margins, round cell subtype, and pleomorphic subtype were associated with significantly decreased disease-specific survival (log-rank test p = .048, .041, .021, and .012, respectively). CONCLUSIONS: Based on this limited experience and existing literature, we continue to recommend surgery with negative margins as the treatment of choice and that adjuvant therapies should be considered in patients with high-grade histology, large tumors, positive margins, or certain subsites.

Intraoperative high-dose-rate brachytherapy (IBT) for locally unresectable intraabdominal malignancy.

PURPOSE: Intraoperative high-dose-rate brachytherapy (IBT) has been successfully used in locally advanced unresectable intraabdominal malignancy. We retrospectively evaluated the safety, feasibility, and general outcome of IBT following cytoreductive surgery. PATIENTS AND METHODS: After radical resection, the target area to be treated by IBT was determined jointly by the surgeon and the radiation oncologist. A silicon template was used to position parallel hollow catheters spaced 1 cm apart against the area of interest. IBT doses were prescribed at 1 cm depth from the template surface and calculated using standard plans. Radiation was administered in a dedicated shielded room. RESULTS: Between August 2001 and February 2006, 10 patients (colorectal cancer n = 6, cervix cancer n = 1, extramedullar plasmocytoma n = 1, liposarcoma n = 1 and sacrococcygeal teratocarcinoma n = 1) were treated. The mean delivered IBT dose was 8 Gy (range 7.5-20). No postoperative mortality was seen, while major complications developed in one (10%) patient with a rectovaginal fistula and intraabdominal abscess. Five of the six colorectal cancer patients developed local recurrence while 3 also developed distant metastases. The mean disease-free and overall survival in this group was 8.5 months (range 4-15) and 25.5 months (range 10-48) respectively. Palliation of symptoms was observed in 89 % of cases. CONCLUSION: IBT combined with debulking surgery is feasible and can be safely performed. While cure is rarely achieved, IBT offers the potential to prolong local control and survival in locally unresectable intraabdominal cancer. Therefore, IBT can be considered as a valuable adjuvant in the therapeutic and palliative armamentarium in these selected patients.

Primary cardiac liposarcoma causing cardiac tamponade: report of a case.

The intracardiac growth and extension of liposarcoma was observed in a 60-year-old woman. The epicardial tumor was identified to originate from the anterior wall of the right ventricle. She initially showed symptoms associated with cardiac tamponade. A surgical operation was performed but it resulted in incomplete resection due to massive invasion and dissemination. The recurrence of the tumors led to congestive heart failure. Finally, she died of heart failure and liver dysfunction as a result of tumor metastasis and invasion. An autopsy detected the primary cardiac liposarcoma. Only a few cases of cardiogenic liposarcoma have so far been reported. A further elucidation of cardiac liposarcoma could reveal mechanisms of the disease, and thus contribute to development of complementary therapies after surgical intervention.

Liposarcoma retroperitoneal gigante / Giant retroperitoneal liposarcoma

El liposarcoma es un tumor maligno de origen mesodérmico derivado del tejido adiposo y el más frecuente de los sarcomas de partes blandas de localización retroperitoneal. Puede alcanzar enormes proporciones. Es de crecimiento lento y su síntoma más frecuente es el dolor abdominal inespecífico y aumento del perímetro abdominal. El tratamiento es la cirugía radical y su resección completa es esencial para el control local de la enfermedad. Presentamos un caso de liposarcoma retroperitoneal gigante derecho bien encapsulado que pudo ser extirpado en su totalidad. El paciente se encuentra en seguimiento evolutivo, pasados 2 años, y libre de enfermedad (AU)

Liposarcoma is a malignancy of fat cells and is the most frequent soft tissue sarcoma localized in the retroperitoneum. It can reach substantial proportions. It is a slow-growing tumor, and the most frequent symptom is nonspecific abdominal pain and diffuse abdominal enlargement. Treatment is radical surgery and complete resection is essential for local control of the disease. We present a case of giant right (..) (AU)

Hyperthermia treatment planning and temperature distribution reconstruction: a case study.

While a great deal of effort has been applied toward solving the technical problems associated with modelling clinical hyperthermia treatments, much of that effort has focused on only estimating the power deposition. Little effort has been applied toward using the modelled power depositions (either electromagnetic (EM) or ultrasonic) as inputs to estimate the hyperthermia induced three-dimensional temperature distributions. This paper presents a case report of a patient treated with hyperthermia at the Duke University Medical Center where numerical modelling of the EM power deposition was used to prospectively plan the treatment. Additionally, the modelled power was used as input to retrospectively reconstruct the transient three-dimensional temperature distribution. The modelled power deposition indicated the existence of an undesirable region of high power in the normal tissue. Based upon this result, amplitudes and phases for driving the hyperthermia applicator were determined that eliminated the region of high power and subsequent measurements confirmed this. The steady-state and transient three-dimensional temperature distributions were reconstructed for four out of the seven treatments. The reconstructed steady-state temperatures agreed with the measured temperatures; root-mean-square error ranged from 0.45 to 1.21 degrees C. The transient three-dimensional tumour temperature was estimated assuming that the perfusion was constant throughout the treatment. Using the computed three-dimensional transient temperature distribution, the hyperthermia thermal dose was computed. The equivalent minutes at 43 degrees C achieved by 50% (T50Eq43) of the tumour volume was computed from the measured data and the three-dimensional reconstructed distribution yielding T50Eq43 = 40.6 and 19.8 min respectively.

[A case of retroperitoneal liposarcoma].

A 77 year-old woman presented with the chief complaint of a mass in the right upper quadrant. The examination on admission including IVP, echography, Ga scintigraphy, barium enema, CT scan, and angiography revealed that the right kidney and ascending colon were displaced by the mass. Under the diagnosis of retroperitoneal tumor, operation was carried out. Most of the mass was resected with the right kidney. The pathological diagnosis of the resected tumor revealed well differentiated liposarcoma. Convalescence was uneventful and the patient has been well and free from disease for 14 months. Based on our experience and the review of the pertinent literature in English and Japanese, we emphasize the importance of definite surgery and close follow up, as liposarcoma is frequently recurrent.