Childhood rhabdomyosarcoma

Overall five-year survival of Childhood rhabdomyosarcoma (RMS) is reported to be 70% in the Intergroup Rhabdomyosarcoma Study Group (IRSG), however, the figure in Japan is almost 15% lower than that of IRSG. Treatment regimen of RMS essentially depends on the histology of the tumor, site, preoperative staging and postoperative grouping that leads to the risk classification. VAC is a standard chemotherapeutic regimen for low and intermediate risk group. Clinical trial with CPT-11 or high dose chemotherapy are underway for high risk RMS.     

Childhood rhabdomyosarcoma in otorhinolaryngology

Two cases of childhood rhabdomyosarcoma with otorhinolaryngological localization are reported and the literature of this rare malignancy is reviewed. The prognostic importance of localization, early diagnosis and combined surgical, irradiation and cytostatic treatment are emphasized.     

Normal and abnormal development of the extremities and trunk

Normal and abnormal embryologic and postnatal development of the extremities, chest wall, breasts, and abdominal wall are described. Knowledge of development anatomy is helpful in forming a rational basis for planning and timing of surgical intervention. Clinical and potential clinical applications are described.     

Tissue expansion in functional and aesthetic reconstruction of the trunk and extremities. Case report.

To illustrate how the technique of tissue expansion may be adapted for different types of reconstruction, the following cases are described: a 10-year-old girl with a circumferential naevus from shoulder (at the back) and umbilicus (at the front) to the knees; a 3-year-old boy with a severe injury to the left arm; a 36-year-old man with an amputation injury of the left hip; and an 18-year-old girl with scars from a complicated fracture of the left lower leg. In all cases function and/or appearance were considerably improved.     

An analysis of 114 patients with dermal melanocytosis on the trunk and extremities.

Dermal melanocytosis is commonly treated with Q-switched neodymium:yttrium-aluminium-garnet (Nd:YAG) laser today. We report on the clinical effect of laser treatment of dermal melanocytosis of the trunk and extremities in 114 patients. The effect of numbers of such treatment was assessed by clinical examination and classified into six grades. All the patients who had more than eight treatments achieved more than 75% clearance. Deep-blue with brown speckled lesions tended to clear more easily than uniform deep-blue lesions. We expect that all dermal melanocytosis of the trunk and extremities can be cleared after a maximum of 12 treatments.     

Soft tissue sarcomas of the extremities and trunk in the adult

Summary Between 1970 and 1988 surgery was performed on 124 patients with soft tissue sarcomas of the extremities and trunk in the University Clinics of Hamburg. Liposarcoma, malignant fibrous histiocytoma, fibrosarcoma and malignant schwannoma were the most common histological types. High-grade sarcomas (G3) predominated, with 41 %, while 26% were graded G2 and 33% G1. Resection with wide margins all round was achieved in only 54% of the patients. The quality of the operation proved to be only therapy-related prognostic factor. In addition, tumour grade, size, regional lymph node and distant metastasis and histological type proved to be relevant to the prognosis. With multivariate analysis, distant metastasis, grade, resectability and histological type retained prognostic significance. The efficacy of adjuvant chemo- and radiotherapy was related to the quality of the preceding tumour resection. In case of gross tumour the rate of either partial or complete response was 28% for chemotherapy and 22% for radiotherapy. The mean survival time was 102 months; the 5- and 10-year survival rates were 48% and 37%, respectively.

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Weichteilsarkome der Extremitäten und des Rumpfs bei Erwachsenen. Bericht von 124 Fällen

Zusammenfassung In der Chirurgischen Universitätsklinik Hamburg wurden von 1970–1988 124 Patienten mit Weichteilsarkomen der Extremitäten und des Rumpfs operiert. Liposarkome, maligne fibröse Histiozytome, Fibrosarkome und maligne Schwannome waren die häufigsten histologischen Typen. Niedrig differenzierte Sarkome (G3) überwogen mit einem Anteil von 41%, während 26% als G2 und 33% als G1 imponierten. Bei nur 54% der Patienten wurde eine Tumorresektion mit dreidimensional weitem Sicherheitsabstand durchgeführt. Dabei stellte sich die Qualität der Operation (RO/1/2) als einziger im Rahmen der Therapie beeinflu\barer Prognosefaktor heraus. Daneben wurde in der univariaten Analyse das Tumorgrading, die Tumorgröße, die regionalen Lymphknoten- und Fernmetastasen und der histologische Typ als prognostisch relevant ermittelt. In der multivariaten Analyse behielten die Faktoren Fernmetastasen, Grading, Resektabilität und histologischer Typ statistische Signifikanz. Der Erfolg einer adjuvanten Chemo- und Strahlentherapie war erheblich von der Qualität der vorangegangenen Tumorresektion abhängig. Bei klinisch manifestem Tumor wurde mit einer Chemotherapie bei 28% der Fälle eine Voll- oder Teilremission erreicht, mit einer Strahlentherapie bei 22% der Fälle. Die durchschnittliche Überlebenszeit betrug 102 Monate, die 5- und 10-Jahres-Überlebensrate 48% bzw. 37%.

     

Sentinel lymph-node biopsy for melanoma of the trunk and extremities: the McGill experience.

OBJECTIVE: To determine the effectiveness of sentinel lymph-node (SLN) biopsy for melanoma of the trunk and extremities. DESIGN: Case series review. SETTING: Royal Victoria Hospital, a Canadian university hospital. PATIENTS: Thirty-six patients (18 women and 18 men) seen between October 1996 and December 1998 with melanoma 1 mm or more in thickness with clinically negative lymph-node basins. Follow-up was 396 days. INTERVENTIONS: SLN biopsy. Technetium-99m filtered sulfur colloid (0.5 mCi) was injected intradermally around the melanoma or the excision scar 10 to 15 minutes before the surgical skin preparation. The identification of the SLN(s) was done with a hand-held gamma probe. Local anesthesia was used mostly for inguinal SLN biopsy whereas general anesthesia was usually required for axillary SLN biopsy. Preoperative lymphoscintigraphy was used only for trunk melanomas. OUTCOME MEASURES: Morbidity, successful identification of the sentinel node and locoregional recurrence. RESULTS: The mean age of patients at diagnosis was 53.4 years (range from 22-76 yr). The melanomas were distributed between the lower extremities (20 patients), upper extremities (8 patients) and trunk (8 patients). The mean Breslow thickness was 2.35 mm (range from 1-8 mm). Lymphoscintigraphy accurately localized the lymph-node drainage basin for trunk melanomas. In 1 patient the SLN could not be identified because the radiocolloid failed to migrate (failure rate 2.8%). The average number of SLNs removed was 1.97. Eight patients (22%) had sentinel nodes positive for malignant disease. The postoperative complication rate was 8.5%. Seven of 8 patients with positive SLNs underwent a complete node dissection (1 patient refused). Of the completion dissections only 2 patients had positive non-SLNs. All patients with positive nodes received interferon alpha-2b as adjuvant treatment. At follow-up, 34 patients are alive with no evidence of disease, 1 patient with a positive SLN is alive with distant metastatic disease and 1 patient with a negative SLN is dead of disseminated disease. CONCLUSION: SLN biopsy is a feasible technique with an acceptable failure rate and is thus a useful tool in the surgical management of melanoma.     

Survival in primary soft tissue sarcoma of the extremities and trunk

Background: Soft tissue sarcomas (STS) of the extremities are rare. The purpose of this study was to identify prognostic risk factors associated with survival in patients with primary extremity and truncal STS.Methods: Patient, tumor, and pathologic data from 149 consecutive patients with localized primary STS of the extremities and trunk were analyzed using Kaplan-Meier and Cox regression techniques to identify univariate and multivariate risk factors. A subgroup analysis was performed to compare factors predictive of survival in patients who received treatment before (n=50) and after (n=99) treatment was standardized in 1988.Results: The 5-year survival rate was 76.5% with an average follow-up of 6 years. Local recurrence occurred in 23% of all patients, 40% before 1988 and 15% after 1988 (P<0.0001). Risk factors associated with survival included resection quality (R0 vs. R1;P<0.0001), era of operation (P=0.002), local recurrence (P<0.001), UICC stage (P<0.0001), tumor size (P<0.001), tumor depth (P=0.002), regional lymph nodes (P<0.0001), and histology (P<0.0001). Multivariate analysis revealed that tumor size, tumor depth, and resection quality were independent risk factors of survival.Conclusions: These results indicate that management of STS in a specialized institution improves overall survival. Resection quality is the most important risk factor of survival. Therefore, effort should be made during primary treatment of STS to achieve wide, tumor-free resection margins.     

Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities

Purpose

The aim of this study is to investigate a new treatment regimen for macrocystic and microcystic lymphatic malformations (LMs) of the trunk and extremities.

Methods

Sixteen patients (aged 2 months-22 years) underwent percutaneous treatment for LM of the trunk and extremities from 2002 to 2007. The LM involved the arm, leg, axilla, chest, abdomen, scrotum, and penis. Eleven patients underwent primary treatment of LM; 5 were treated for recurrence after prior operative resection. Macrocysts (≥1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline was used for microcysts. Macrocysts and microcysts were treated after complete cyst aspiration using sonographic guidance. Twenty-four macrocysts and 103 microcysts were treated. The goal of treatment was complete cyst ablation documented by sonography or magnetic resonance imaging.

Results

The mean number of treatments was 1.7 per patient; the mean number of treatments for macrocysts was 1.3 and for microcysts was 1.7. Ablation efficacy was 100% (127/127 cysts). Treatments included massive intraperitoneal cysts and cysts surrounding the adventitia of the brachial artery and brachial nerve. Infection occurred in 2 (13%) of 16 patients. No patient experienced postprocedural pain, skin necrosis, neuropathy, bowel obstruction, skin retraction, or myoglobinuria.

Conclusions

Percutaneous sclerotherapy provides effective treatment for macrocystic and microcystic LM as primary treatment or for recurrence after surgical resection.     

Malignant fibrous histiocytoma of the extremities and trunk: an institutional review

BACKGROUND: Malignant fibrous histiocytoma (MFH) is the most common subtype of soft-tissue sarcoma. Detailed understanding of this tumor type may lead to improved therapeutic strategies. METHODS: An institutional review was performed on all patients with primary MFH of the extremities and trunk operated on between 1988 and 2000. RESULTS: Ninety-seven patients with histologically confirmed MFH (G1, n=8; G2, n=25; G3, n=64) were analyzed. Local recurrence was 31% after a median of 13 months. Distant metastases occurred in 29 patients (30%). After a median follow-up of 4.5 years, 54 patients were alive without evidence of disease; median survival time was 84 months at a cumulative 5-year survival rate of 70%. Tumor size significantly influenced disease-free survival (T2 vs T1, P<.01, risk ratio [RR] 6.0), as did tumor depth (subfascial tumors, P<.01, RR 3.1) and presence of positive lymph nodes (P=.02, RR 6.9). Positive microscopic margins and subfascial tumors were associated with an increased local recurrence rate (RR 4.8, P<.001 and RR 3.5, P=.02, respectively). Significant multivariate risk factors of distant metastases were tumor size, depth, and grade. Though not performed in a randomized fashion, a subgroup analysis indicated that adjuvant radiation therapy significantly reduced local tumor failure. CONCLUSION: We conclude that aggressive, albeit limb-preserving resection of MFH, should be performed at initial operation to minimize risk of local recurrence; a strict follow-up especially of subfascial tumors should be performed.     

Desmoid tumours of the extremities and trunk: a review of the literature

Desmoid tumours are rare neoplasms that display local aggressiveness but no propensity to metastasise. They are mainly localized in the abdominal wall, the bowel, and the mesentery or in extra-abdominal sites such as the trunk and the extremities. Surgical resection is the main treatment modality in extremities and trunk, with the optional combination of radiotherapy and/or chemotherapy. However, these tumours have a high propensity for recurrent growth.

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Résumé Les tumeurs desmoides sont des néoplasmes rares qui ont un caractère agressif local, mais aucune propension à métastaser. Elles sont principalement localisés dans la paroi abdominale, lintestin et le mésentère et dans des sites extra-abdominaux tels que le tronc et les extrémités. La résection chirurgicale est la principale modalité du traitement pour les extrémités et le tronc avec la combinaison facultative de radiothérapie et/ou chimiothérapie. Cependant, ils ont une haute tendance à la récidive.

     

Management of soft tissue infections in the region of the extremities and the trunk

Skin and soft tissue infections (SSTI) are amongst the most common bacterial infections in humans. SSTI have a broad range of aetiology, clinical manifestation and severity. The outcome may be spontaneous resolution or on the other end sepsis with lethal outcome. Useful classifications are those which differentiate SSTI according to urgency of surgical intervention. The definitive diagnosis should be made by the clinical picture of the lesion and the condition of the patient. The key to successful treatment of many severe necrotizing soft tissue infections is based on early detection and prompt surgical debridement. This procedure has to be followed by an optimal wound management. From the early beginning of treatment an adequately calculated antibiotic treatment is mandatory. At the end of the treatment the wound has to be closed by an aesthetic scar with high mechanical load capacity.     

警惕下肢内固定术中主干动脉损伤

下肢骨折内固定术中动脉损伤很容易漏诊和延误治疗,并造成严重残废[1].2002年3月～2005年3月,笔者共收治4例下肢内固定术中并发主干动脉损伤病例.现报道并分析原因,旨在提高对这种损伤的警惕,最大限度地避免该并发症的发生.     

Preventive lymphadenectomy in complex treatment of melanoma of the skin, trunk and extremities

Results of treatment of 90 patients with primary melanoma of the skin, trunk and extremities with stages T2-T4N0M0 were analyzed. Different strategies were used in relation to regional lymph nodes. It was found that prophylactic lymphadenectomy in terms of more than 4 weeks after excision of primary cutaneous melanoma reliably prolongs total and recurrence-free survival as compared with the method without fulfilling prophylactic lymphadenectomy. Prophylactic lymphadenectomy fulfilled in terms less than 4 weeks fails to have statistically significant influence on the results of treatment.     

A proposed new system of coding and injury classification for arteries in the trunk and extremities

Study designRetrospective review of arterial injury with prospective intra-observer and inter-observer analyses.AimTo introduce a new classification system for arterial injuries in the extremities and trunk.MethodsRetrospective review of all patients with arterial injuries treated at a level I trauma centre during a 3.5-year period. Major arteries were located and coded, and the arteries’ lesion was categorised in reference to the alphanumeric format of the AO fracture coding system and injury classification. Each major artery was coded in a numeric format and further divided into three segments: proximal, middle and distal. The severity of artery injury was classified into different types and subtypes in an alphanumeric format. Inter-observer and intra-observer reliabilities were tested.ResultsSystemic arteries were coded and arterial injuries were classified in the alphanumeric notation. The intra- and inter-observers’ reliabilities were found to be acceptable in the application of the new system.ConclusionThe preliminary data demonstrate that the new coding and injury classification system for arteries in the trunk and extremities are reliable and efficient for data storage and retrieval, and provide a favourable environment for discussion among different physicians or centres.     

Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma.

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.