Stereotactic radiosurgery in alveolar soft part sarcoma brain metastases: Case series and literature review.

Alveolar soft part sarcoma (ASPS) has the highest incidence of brain metastasis amongst sarcomas. There is a paucity of literature published focusing on radiation therapy for this condition. This is a single centre retrospective review of the treatment of three patients with 12 ASPS brain metastasis using single dose stereotactic radiosurgery (SRS). Five lesions were treated with low (<25 Gy) and seven with high (≥25 Gy) dose. Four lesions had a volume of >1.5 cm3 and were defined as large, while seven had a volume of ≤0.5 cm3 and were defined as small. The local tumor control as well as the clinical complication rates were studied. There was a statistically significant relation between treatment dose and tumor control rate. All large tumors treated with low dose recurred and required surgical removal within two months following SRS, while the large lesion treated with high dose recurred after 11 months. Five of the six small tumors treated with high doses were controlled, while the sixth required retreatment and was stable thereafter. No patient suffered from undue symptomatic radiation effects. The success rate following SRS for small ASPS metastases treated with high doses seems to be sufficient to justify the treatment. The short time for large tumor to recur, significant increase in tumor size requiring surgical removal of the tumors, makes low dose SRS unattractive. Based on this limited patient population, it seems that high dose SRS should be used for all ASPS brain metastases except for large tumors deemed surgically accessible.

Alveolar soft part sarcoma of the right calf: A case report.

RATIONALE: Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue neoplasm with controversial histogenesis. ASPS accounts for 0.5% to 1% of all soft tissue sarcomas. Because of its rarity, ASPS is easily misdiagnosed, increasing the risk of incorrect treatment. PATIENT CONCERNS: A 6-year-old female patient presented with a history of a 2.0 × 2.5 × 3.0-cm mass in the deep soft tissues of her right lower extremity. DIAGNOSES: Histopathological features indicated the diagnosis of ASPS. Microscopically, a diffuse arrangement of tumor cells or pseudoalveolar architectures separated by thin and well-vascularized fibrous septa were observed. Immunohistochemical staining of the tumor cells indicated positivity for transcription factor E3, myogenic determination factor 1, and periodic acid-Schiff-diastase (PAS-D) and showed a Ki-67 proliferating index of approximately 20%. INTERVENTIONS: The patient underwent enlarged resection of the tumor and was treated with radiotherapy. OUTCOMES: During the 3-year follow-up, the patient has remained in good condition, with no symptom recurrence, distant metastatic spread, or significant toxicity during or after treatment. The patient remains under regular surveillance. LESSONS: Its low incidence, lack of characteristic clinical manifestations, and atypical location often lead to ASPS misdiagnosis and subsequent incorrect treatment. Nuclear expression of transcription factor E3 is of diagnostic value for ASPS. At present, there is no consensus on the treatment for ASPS. In-depth pathological analysis is needed to better understand the characteristics of this tumor.

Anticipated intraoperative electron beam boost, external beam radiation therapy, and limb-sparing surgical resection for patients with pediatric soft-tissue sarcomas of the extremity: a multicentric pooled analysis of long-term outcomes.

PURPOSE: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. METHODS AND MATERIALS: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. RESULTS: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). CONCLUSIONS: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results.

Management of recurrent alveolar soft-part sarcoma of the tongue after external beam radiotherapy with iodine-125 seed brachytherapy.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare type of soft tissue sarcoma. The infrequency of ASPS is such that it accounts for <1% of all soft tissue sarcomas and <0.1% of sarcomas concerning the head and neck, primarily those involving the orbit (48%) and tongue (25%). Traditional chemotherapy or radiotherapy of ASPS is often associated with poor outcome, even after comprehensive interventions. METHODS AND RESULTS: We performed iodine-125 ((125) I) seed brachytherapy under ultrasound guidance through a submandibular puncture in a 4-year-old boy with recurrent ASPS of the tongue. The prescription dose was 120 Gy; therefore, 35 (125) I seeds were implanted with 0.77 mCi per seed, the total amount of activity being 26.95 mCi. CT scans confirmed a complete response after the treatment. CONCLUSION: Our patient now has a recurrent-free survival of >30 months, an amount of time longer than the median rate described in the literature.

[Treatment and prognosis of stage IV alveolar soft part sarcoma].

OBJECTIVE: To analyze the clinical features, treatment and prognosis of Stage IV alveolar soft part sarcoma. METHODS: To analyze the clinical and pathological features, therapeutic methods and follow-up results in 21 patients with stage IV alveolar soft part sarcoma. There were 11 males and 10 females, in the age of 26-57 years (average 37.0 years old). All the 21 patients had metastasis: nine cases had multiple pulmonary metastasis, three cases had multiple pulmonary and brain metastasis, two cases had multiple brain metastasis, two cases had multiple pulmonary and bone metastasis, two cases had single pulmonary metastasis, one case had single bone metastasis, one case had single brain metastasis and one case had single soft tissue metastasis. Eight patients were treated by surgical operation, including five cases of complete resection for the primary and (or) metastatic tumor and 3 cases of palliative operation for the primary tumor. All patients received chemotherapy, including seven cases of CAVD regimen and 14 cases of MAID regimen treatment. One patient with single bone metastasis and five patients with multiple brain metastasis received post-operative whole brain radiation therapy. RESULTS: All the eight patients with surgical operation had healing by first intention, and pathological examination showed that seven patients achieved R0 surgical margin and one case with R2 status. One patient with single brain metastasis had recurrence after operation. The toxic and adverse reactions of all patients treated with chemotherapy were tolerable. Among them, 17 cases had stable disease and 4 cases had disease progression after chemotherapy. The disease control rate (DCR) was 81.0%. The DCR of patients with CAVD regimen chemotherapy was 85.7% and that of patients treated with MAID regimen was 78.6% (P = 0.862). All patients were followed up for 8 - 86 months (average 32.4 months). The median survival time of all patients was 32.6 months. The 2-year survival rate was 55.1% and the 5-year survival rate was 21.8%. The median survival time in the patients with complete resection was 60.0 months, and that in patients with palliative operation was 27.0, showing a significant difference between them (P = 0.048). The median progression-free survival in patients with complete excision was 57.2 months and that in patients with palliative operation or without operation was 19.6 months, with a significant difference (P = 0.029). The median survival time in patients who received CAVD regimen chemotherapy was 30.0 months, and that in patients with MAID regimen was 51.0 months, with a non-significant difference (P = 0.511). The median progression-free time in patients with CAVD regimen chemotherapy was 13.0 months, and that in patients with MAID regimen was 38.0 months, also with a non-significant difference (P = 0.066). CONCLUSIONS: Alveolar soft part sarcomas are rarely seen and highly malignant tumors, and the prognosis of stage IV ASPS is poor. Complete resection of all tumors is the key of successful treatment of Stage IV ASPS, and the site and number of tumor metastasis are important factors affecting prognosis. The curative effects of radiotherapy and chemotherapy for ASPS need to be further investigated.

Supracricoid laryngectomy with cricohyoidoepiglotto-pexy or cricohyoido-pexy: experience on 32 patients.

OBJECTIVE: Supracricoid laryngectomy (SCL) with Cricohyoidoepiglotto-pexy (CHEP) or Cricohyoido-pexy (CHP) is an organ preservation surgery indicated for early and selected advanced laryngeal cancers. To verify the clinical usefulness of supracricoid laryngectomy versus total laryngectomy, a retrospective review was conducted. METHODS: We summarized the clinical and postoperative data of 32 patients who received SCL over the past 9 years (1997-2005). Five-year survival rate of the SCL patient group (29 cases) was compared with that of the patient group receiving total laryngectomy (35 cases) within the same period. RESULTS: Wound infection was detected in 12 patients (38%). Those with severe infection, which required surgical intervention, included two cases of ruptured pexis and two cases showing cricoid cartilage necrosis induced by Forestier disease. There were two T4 cases that resulted in extensive excision. In one case, excision involved the posterior part of the cricoid cartilage resulting in insufficient closure of the neoglottis; the patient received total laryngectomy 30 months after SCL-CHEP because of persistent aspiration of liquid diet. In the other T4 case, the tumor invaded the thyroid and arytenoid cartilages but not the cricoid cartilage. Reposition of the remaining corniculate cartilage resulted in sufficient closure of the neoglottis; this patient subsequently acquired satisfactory laryngeal function. The 5-year overall survival rate was 86% for SCL group and 61% for the total laryngectomy group (limited to Stages III and IV glottic cancers). The causes of the four deaths were distant metastasis, neck metastasis, and intercurrent disease, respectively. Two patients are alive with distant disease. CONCLUSION: Through our experience in this series, the functional and oncological results of SCL showed certain advantages over those of total laryngectomy. Particularly, the clinical impact of SCL-CHEP was impressive; this technique needs is recommended to both head and neck surgeons and patients.

[Aggressive course of a malignant alveolar soft tissue sarcoma]. / Aggressive Verlaufsform eines malignen alveolären Weichteilsarkoms.

Alveolar soft part sarcoma is a rare soft tissue tumor. Most patients who are affected by this sarcoma are between 15 and 35 years old. The tumor is characterized by its uncommon location of metastasis. Publications concerning this sarcoma subform are rare and the best therapeutic procedure is not yet clear. Surgical excision, radiation and chemotherapy are performed, whereas complete surgical excision achieves the best results in long-term follow-up. We report a patient's history who rapidly died of his sickness despite the low initial tumor stage.

[Clinical features and prognosis of alveolar soft part sarcoma].

OBJECTIVE: To investigate the clinical feature, suitable treatment and prognosis of alveolar soft part sarcoma. METHODS: The clinical data of 58 such patients treated at our hospital from 1967 to 2006 were retrospectively analyzed. Fifty-three patients except 6 with distant metastasis underwent surgical resection including 33 extensive resection and 19 local resection. Furthermore, 19 patients received postoperative adjuvant radiotherapy or chemotherapy. RESULTS: Eleven of 50 patients who underwent complete resection developed local recurrence. Thirty-One (53.4%) was found to have lung metastasis. The overall 3-, 5-, 10-year survival rate was 89.5%, 74.1% and 57.7%, respectively. The median survival time of the patients was 125 months. The 3-, 5-, 10-year survival rate was 100.0%, 81.6% and 65.3% for female patients, but it was 79.6%, 67.2% and 49.7% for male patients, respectively. CONCLUSION: Alveolar soft part sarcoma usually grows slowly. Though local recurrence is rarely seen, distant metastasis is frequently observed. Lung is the most common organ of metastasis. However, survival can still be long even with lung metastasis. To achieve microscopically complete resection is quite critical to localized alveolar soft part sarcoma. No survival advantage was observed when adding adjuvant radiotherapy and/or chemotherapy. The phenomena of female patient or patient with X chromosome translocation showing better prognosis than the male or other patients needs further investigation.

Alveolar soft part sarcoma of the forearm: a case report.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma that most commonly arises in the deep soft tissues of the lower extremities of adults. Median survival of patients with metastatic ASPS has been reported to be from 3 to 3.3 years. The time between detection of metastases and death varies from 10 months to 6.2 years. In this article, a case of an 11-year-old male with primary ASPS of the right forearm is presented. Successful long-term local control of the primary tumor was achieved with wide margin surgical resection and adjuvant radiation therapy. Three years after diagnosis, the patient developed pulmonary metastases. Chemotherapy was unsuccessfully used to control the metastatic disease. Despite that, the patient survived longer than expected, and passed away 9 years after the detection of pulmonary metastases. This time to death after the development of metastases vastly exceeded the previously reported survival rates of patients with metastatic ASPS.

[Alveolar soft tissue sarcoma]. / Alveoläres Weichteilsarkom.

Alveolar soft part sarcoma is a malignant soft part tumor the etiology of which has not yet been clarified. Histopathological examinations show an alveolar or pseudoglandular pattern. PAS-positive, diastase resistant intraplasmic inclusion bodies are characteristic for this tumor which occurs in the head and neck region; mainly the tongue and the orbita. Primary therapy includes total resection which can later be combined with radiotherapy or chemotherapy. The present study involves a 17 year old patient. We totally removed the tumor and then used radiotherapy. The postoperative examinations over 3 years showed neither local recurrence nor distant metastases.

Radiation therapy for alveolar soft-part sarcoma.

A clinical experience with radiotherapy in 18 patients with alveolar soft-part sarcoma is presented. Adjuvant radiotherapy was associated with prolonged local control in six of six patients without metastatic disease at diagnosis; later one patient relapsed systematically. Meaningful palliation was achieved in all patients with extra-skeletal (and possibly skeletal) metastatic disease. Radiation therapy may be beneficial for patients with alveolar soft-part sarcoma by enhancing local control achieved with limited surgery, by retarding progression of metastatic deposits, and by providing meaningful palliation.