Surgery in rhabdomyosarcoma of the bladder,prostate and vagina

Summary The treatment of bladder and prostate rhabdomyosarcoma (RMS) is highly controversial. Aside from chemotherapy, treatment modalities include conservative surgery, radical surgery, and pre-, intra-, or postoperative irradiation. Between 1968 and 1993, 78 children with RMS were treated at our institution. In all, 22 tumors were located in the urogenital tract (bladder/prostate, 13; paratesticular, 5; vaginal, 2; others, 2). Altogether, 6 patients had stage II disease; 7, stage III disease; and 2, stage IV disease. All 15 patients with RMS of the bladder, prostate, or vagina received chemotherapy, and 4 had additional radiotherapy. Surgery was also done in 10 patients; parents refused an operation in 3 cases. In all, 3 patients in an advanced tumor stage died of their disease. All other children currently show no evidence of disease (mean follow-up, 6 years; range, from 2 months to 18 years). After chemotherapy, radical operative intervention with multiple biopsies (encircling the tumor) — in contrast to local tumor excision or partial resection — permits complete tumor resection followed by excellent long-term results. Following radiotherapy, often a consequence of organ-sparing therapy, many complications ensued (60%); therefore, irradiation should be restricted to highly selected cases.     

Management and prognosis of head and neck sarcomas

Between 1962 and 1988, a total of 104 patients with head and neck rhabdomyosarcoma (RMS) and 17 patients with nonrhabdomyosarcoma (NRMS) were evaluated and treated at St. Jude Children's Research Hospital. All parameningeal sites (middle ear, orbit, or nasopharynx) were excluded from further analysis; thus, 50 patients represent the cohort of head and neck sarcomas for this review. Survival was good in this group of patients, 28 of 50 being alive and disease-free at last follow-up. Twenty of the 38 patients with RMS were alive and disease-free. Similarly, 8 of the 12 patients with NRMS were disease-free at a median follow-up of 5 years. However, the site and size of the primary tumor impacted on the extent of the initial resection and further treatment in addition to surgery. Although the treatment policy evolved over time to a stage-specific strategy for treatment modalities, the data suggest that surgery alone may be sufficient initial therapy for a subset of patients. For patients in whom complete resection is not achieved, the addition of radiotherapy and chemotherapy may result in improved survival.     

Extrapulmonary small cell neuroendocrine carcinoma of the paranasal sinuses: a case report and review of the literature

Primary small cell neuroendocrine carcinoma of the paranasal sinuses is an extremely rare and distinctive tumor with aggressive clinical behavior. We report on a patient who had a small cell neuroendocrine carcinoma of the ethmoid sinus with invasion of the upper aspect of the right maxillary sinus and medial aspect of the right orbit. After 2 cycles of chemotherapy that were unsuccessful, he underwent radiotherapy with complete tumor remission. Local recurrence was not identified after 11 months of follow-up; however, distant metastasis developed 2 months after radiotherapy. The natural history, pathologic features, and choice of therapy for this rare neoplasm are discussed, along with a review of the published data.     

Treatment Strategies for Perianal Rhabdomyosarcoma: Report of Two Cases

Intensive chemotherapy and high-dose radiation with complete excision of the tumor are the treatment of choice for rhabdomyosarcoma (RMS). However, because there are so few reports of perianal RMS, a mainstream treatment has not been established. We report two cases of perianal RMS and review 13 cases documented in Japan since 1990. Case 1 was a 7-year-old boy first treated by tumor excision followed by chemotherapy and external irradiation. He has since been tumor-free but suffered severe perianal erosion and ulceration. Case 2 was a 17-year-old girl first treated by preoperative chemotherapy to reduce the tumor size, after which the tumor was completely excised with anatomical guidance using three-dimensional computed tomography via the posterior sagittal approach, followed by open intraoperative irradiation. She has since been tumor-free with preserved anal function. These case reports show the importance of cogitated treatments for preservation of anal function and optimal therapeutic outcome in patients with perianal RMS.     

Soft-tissue sarcomas of the extremities in patients of pediatric age

Purpose The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.     

Intracerebral granulocytic sarcoma. A case report

Granulocytic sarcoma is a tumor composed of proliferating myeloblastic cells, generally found in the orbit. A brain localization is rare. We report the case of a 11-year-old boy treated in our unit for acute myeloblastic leukemia (AML 4 Eo. FAB). After 21 months of complete remission, he developed headache and facial palsy. The CT scan visualized the presence of two frontal and occipital masses. The spinal tap revealed blastic cells in the CSF. The study of the bone morrow showed medullar relapse. A new medullar and cerebro-meningeal remission was obtained with chemotherapy and radiotherapy. CSF and the bone marrow studies can help avoid stereotaxic biopsy can be avoided in this type of tumor     

Tumor Bed Boost Omission After Negative Re-Excision in Breast-Conservation Treatment

Background We evaluated the necessity of a tumor bed boost after whole-breast radiotherapy for early-stage breast cancer after breast-conserving surgery and negative re-excision. Methods Of patients treated at the Virginia Commonwealth and Tufts Universities with breast-conservation therapy for early-stage breast cancer between 1983 and 1999, 205 required re-excision of the tumor cavity to obtain clear margins and were found to be without residual disease. Adjuvant conventionally fractionated whole-breast radiotherapy was given to a total dose of 50 Gy in 25 fractions. The tumor bed boost was omitted. Results The median follow-up was 98 months (range, 6–229 months). The tumor histological diagnosis was primarily infiltrating ductal carcinoma (183 cases; 89%). Nodal involvement was documented in 49 cases (24%). There were four documented recurrences at the tumor bed site. Five in-breast recurrences were documented to be in a location removed from the tumor bed. The overall Kaplan-Meier 15-year in-breast control rate was 92.4%, and the freedom from true recurrence rate was 97.6%. Conclusions The findings support the concept that postlumpectomy radiotherapy can be tailored according to the degree of surgical resection. There is an easily identifiable subgroup of patients who can avoid a tumor bed boost, thus resulting in a reduced treatment time and improved cosmesis, while maintaining local control rates that approach 100%. The data suggest that in patients who undergo a negative re-excision, treatment with whole-breast radiotherapy to 50 Gy is a sufficient dose to maximally reduce the risk of local recurrence.     

原发性骶骨肿瘤的诊断和多学科综合治疗

目的 掌握原发性骶骨肿瘤的诊断依据，评估多学科综合治疗的效果。方法 回顾64例骶骨肿瘤的临床资料，了解原发性骶骨肿瘤的特点，分析手术步骤和引起并发症的原因及预防措施，并对多学科综合性治疗的结果进行随访分析。结果 根据临床症状、体征及影像学改变可作初步诊断，肛门指诊可帮助诊断，结合穿刺活检及病理检查可明确诊断。53例获得随访，无术中死亡，术后5年内死亡9例(16．9％)，术后复发17例(32．07％)，术后发生尿失禁、直肠损伤各3例。经过化、放疗的28例恶性骶骨肿瘤患者术后5年生存率达到了89．2％，而未做相应治疗的20例患者术后5年生存率为52．8％。53例中44例功能结果满意(83．01％)。结论 肛门指诊可协助诊断；全身状况较好的原发性骶骨肿瘤为手术适应证；术后复发与手术不彻底有关；化疗和放疗对不同的骶骨肿瘤有一定的疗效；综合治疗是治疗骶骨肿瘤的方向。     

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery

OBJECTIVE: To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS: From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients. RESULTS: After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation. CONCLUSION: Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.     

Irradiation therapy for gallbladder carcinoma: recent advances

Abstract. Purpose: Gallbladder carcinomas were usually considered to be radioresistant. So far, the role of radiotherapy has not been adequately evaluated. The aim of this report is to assess the value of radiotherapy in carcinoma of the gallbladder. Methods: We reviewed publications concerning the role of radiation therapy in gallbladder carcinoma from 1974 to 2000. External radiation therapy, intraoperative radiation therapy, and brachytherapy were evaluated in two different groups: one group of patients underwent surgery, with apparently complete resection of the tumor; and another group underwent palliative treatment. Results: Local control of the tumor and reduction of tumor size were reported in several publications. Collected data suggested a slight improvement in survival after adjuvant or palliative radiotherapy. The best benefit was obtained in tumors resected with only microscopic residual tissue. If possible an intraoperative "boost" (15 Gy) is recommmended on the gross lesion, residual lesion, or tumor bed. Additional postoperative external radiotherapy (45–50 Gy) must be delivered. Conclusion: Radiotherapy appears to be a safe procedure that slightly improves the survival time of patients treated for gallbladder carcinoma. Further trials are needed to assess the role of combined radiotherapy and chemotherapy. Received: January 9, 2001 / Accepted: August 1, 2001     

Extent of surgery in rhabdomyosarcoma of urogenital structures

After high inguinal semicastration in group-1 paratesticular rhabdomyosarcoma (RMS), the patient having undergone chemotherapy can be followed closely by CT scanning without retroperitoneal lymphadenectomy. In contrast, retroperitoneal RMS should be operated on as radically as possible after downstaging the tumor mass. In RMS of the female genitalia locally limited organ-preserving surgery is the method of choice. The prognosis is excellent with adjuvant chemotherapy. Only 20% of all bladder RMS arise from the bladder dome or the movable part of the bladder, where primary partial resection including a safety margin of 3 cm of healthy tissue is possible. The majority, however, arising from the submucous tissue of the bladder base, trigonal area and bladder neck, infiltrates the prostatic urethra and the surrounding pelvic fascia. Therefore differentiation between primary bladder or prostate RMS is often doubtful. The current strategy of downstaging by chemotherapy or chemo-radiotherapy followed either by limited organ-preserving surgery or by a wait-and-see policy includes the high risk of residual tumor or local tumor recurrence, which has proved to have the worst prognosis of all. As organ-preserving therapy was recommended as the method of choice, mainly to avoid anterior pelvic exenteration and urinary diversion, the question of advantages in comparison to the risk of tumor recurrence is still open. The routine use of chemoradiotherapy and increasing follow-up has called attention to a variety of chronic sequelae, such as contracted bladders with reduced capacity and upper urinary tract deterioration, which subsequently require secondary urinary diversion. Progress in continent urinary diversion may be an alternative procedure for better life quality.(ABSTRACT TRUNCATED AT 250 WORDS)     

Therapeutische Strategien bei lokoregionalen Rezidiven gastrointestinaler Tumoren

Locoregional recurrence is a relevant problem in surgical oncology. Intraluminal local relapse only occurs when the primary resection is carried through with inappropriate safety margins. If possible, a second surgical resection with wider margins is the treatment of choice. When the primary resection was appropriately done, extraluminal relapse in the original tumor bed indicates the primary tumor was already in an advanced stage. The indication for a second resection must be considered carefully. A realistic prospect of long-term tumor control exists only when the second resection yields tumor-free margins and is combined with chemo- and radiotherapy. There is usually no curative treatment option for recurrences in the draining lymph node region, the so-called fourth dimension.     

Orbital malignant fibrous histiocytoma with extension to the base of the skull--case report

An 18-year-old woman presented with a malignant fibrous histiocytoma (MFH) originating in the orbit and invading the frontal and temporal base of the skull manifesting as exophthalmos and double vision that had persisted for 2 months. Magnetic resonance imaging revealed a tumor in the left orbit that extended as far as the frontal and temporal base of the skull. The tumor was treated by radical resection with conservation of the eyeball and its contents, followed by orbit wall reconstruction. The histological diagnosis was MFH. Local radiotherapy was administered postoperatively. The preoperative symptoms improved, and there has been no evidence of local recurrence or metastasis in the year since the surgery. In this case, radical resection of the tumor was essential. Furthermore, the adjuvant therapy was apparently successful, probably because this histological type of tumor is highly sensitive to radiotherapy.     

小儿生殖道横纹肌肉瘤3例并文献复习

目的探讨小儿生殖道横纹肌肉瘤的临床特征、治疗方法及预后情况。方法对我院1985年11月至2008年7月间收治的小儿生殖道横纹肌肉瘤(RMS)患者进行回顾性分析,并复习相关文献。结果小儿生殖道RMS患者3例,占同期小儿妇科恶性肿瘤的5.4%(3/56)。年龄1～5岁。阴道RMS 2例,盆腔RMS 1例;按国际横纹肌肉瘤协会(IRS)分期均为Ⅲ期。初期均进行保守性手术,并联合化疗,均达到完全缓解。2例阴道RMS分别随诊30、13个月无复发。1例盆腔RMS初期治疗停化疗1年复发,予复发病灶切除,并行联合化疗,完全缓解6个月,6个月后出现肺转移,放弃治疗后死亡。结论生殖道是小儿RMS的常见部位,阴道RMS最为常见,强调综合治疗,保守性手术是可行的。预后取决于肿瘤的组织学亚型、大小、病变范围、初始部位、治疗后残留病灶情况及年龄。     

Primitive neuroectodermal tumor arising 8 years after chemotherapy and radiotherapy for acute lymphoblastic leukemia: case report

We report a case of primitive neuroectodermal tumor (PNET) arising 8 years after chemotherapy and radiotherapy for acute lymphoblastic leukemia. A 15-year-old boy with a history of acute lymphoblastic leukemia, at the age of 7, underwent chemotherapy and 14Gy of radiotherapy to the whole brain. He was admitted to our department due to the development of aphasia, right hemiparesis and generalized convulsive seizure. MRI showed an irregularly enhanced mass in the left frontal lobe. A gross total removal of the tumor was performed and histological examination showed it to be PNET. Postoperatively, the patient underwent 20Gy of radiotherapy to the whole brain and 42Gy of local radiotherapy. Follow-up MRI showed no evidence of recurrent tumor 4 months after the radiotherapy. This tumor was thought to be a secondary brain tumor arising in this survivor of childhood acute lymphoblastic leukemia and it is a rare complication of successful leukemia treatment.     

Cyst formation following local chemotherapy of malignant brain tumor: a clinicopathological study of two cases]

This paper reports clinicopathological findings concerning an enlarged bulky cyst and the tumor cavity following local administration of an anticancer agent combined with radiotherapy in two patients suffering from malignant glioma. Case 1: This 69 year-old man who had been diagnosed as having glioblastoma in the right parietal lobe had received local chemotherapy after the first operation. Simultaneously radiotherapy of 69 Gy in total dose was performed. At the second operation for the tumor, cyst formation was clinically confirmed and necrotomy as well as evacuation of the large cyst was performed after adjuvant therapy. The patient died at a time ten months after the first surgical operation. Case 2: This is the case of a 48 year-old man who was diagnosed as having gemistocytic astrocytoma in the left frontal lobe. The first surgical operation was performed and was followed by local chemotherapy as well as radiotherapy (total dose of 90 Gy in two sessions). The second surgical operation of the recurrent tumor, with necrotomy and evacuation of the large cyst were performed after adjuvant therapy. The patient expired at a time sixty-five months after the first surgical operation. Relevant to the chemotherapy, adriamycin (ADM) (0.5mg) and methotrexate (MTX) (1mg) were administered through the Ommaya's reservoir into the tumor bed at craniotomy. The usual doses of ADM and MTX amounted to 5.0mg respectively. Through conventional CT and MRI, formation of a cyst including abundant membranous debris or septi was identified in both cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Laparoscopic insertion of pelvic tissue expander prior radiotherapy for sacral metastasis of alveolar maxillary rhabdomyosarcoma to prevent radiation enteritis

IntroductionRhabdomyosarcoma is the most common soft tissue sarcoma seen in childhood and adolescence. The most frequent sites are head and neck.Presentation of caseA young female with maxillary rhabdomyosarcoma involving region of maxillary sinus with skeletal metastases was primary treated acccording to RMS 2005 protocol. She received 9 cycles of chemotherapy. Primary tumor of maxillary sinus was surgicaly removed after 4 cycles of chemotherapy, with 6th cycle of chemotherapy a radical radiotherapy of primary tumor location and metastasis in spinal vertebras, ribs, pelvic bone and left femoral bone started what leads to complete regression of skeletal metastases. In course of maintenance therapy MRI scan showed 12 × 28 × 23 mm lesion in sacrum in the vicinity of right sacroiliacal joint with caracteristics of metastasis. Because the region of right sacroiliacal joint with bowel was already included in primary radiation treatment, tissue expander was laparoscopicaly inserted in lower pelvis to displace bowel loops from radiation field to prevent radiation enteritis. After external beam radiotherapy to her sacrum, a good response without any side effects was achieved.DiscussionLaparoscopic insertion of pelvic tissue expander prior EBRT and it's subsequent removal after EBRT is safe and effective method for displacing loops of bowel out of the pelvis. With minimal morbidity converts untreatable disease to treatable by allowing delivering high doses of radiation to the patient.ConclusionAfter 2 years of follow up the disease is in remission and the patient without any major complaint.     

The impact of delayed surgery on radiotherapy dose and local control of rhabdomyosarcoma

To determine if delayed surgery permits the modification of radiotherapy dose while maintaining local control in children with localized, unresectable rhabdomyosarcoma, a prospective study was launched in 1981 to test this objective. Treatment consisted of 16 weeks of preoperative chemotherapy, with or without delayed surgery, and radiotherapy using 35 to 40 Gy (3500 to 4000 rad) for microscopic and 50 to 55 Gy (5000 to 5500 rad) for gross residual tumor, plus 14 months of chemotherapy. Among 22 patients treated, surgery was feasible in 11 of 14 patients with residual tumor after chemotherapy and was performed in eight (avoiding radical surgery in three), leaving microscopic (seven patients) or gross residual (one patient) tumor. Progressive disease or amputation precluded radiotherapy in two patients. After radiotherapy local control was sustained in 12 of 14 patients with microscopic lesions vs none of six patients with gross tumor. Delayed surgery may permit the use of lower-dose radiotherapy and should be considered in the treatment plan for this subset of patients.     

Chondrosarcoma of the ethmoid sinus extending to the anterior fossa

A 27-year-old women with chondrosarcoma of the ethmoid sinus extending to the anterior fossa had initial symptoms of blurred vision on the left and slight proptosis. Neuroradiological examination, however, disclosed that the tumor extended not only to the anterior cranial fossa, but also to the orbit, nasal cavity, and maxillary sinus. It is difficult to predict the biological behavior of chondrosarcoma by means of histological examination. The effectiveness of radiotherapy, chemotherapy, or both is also a matter of controversy. Clinical aspects of chondrosarcoma are briefly discussed.     

Primary cranial Ewing's sarcoma

Primary Ewing's sarcoma of the cranium is extremely rare, with only 17 cases reported so far. We describe a further case with involvement of the frontotemporal region and the orbit. The patient, an adult male, was admitted with headache and papilloedema, and later developed localised swelling and proptosis. He was treated with surgery, radiotherapy and chemotherapy, and was free from metastases during a follow-up of 14 months. The prognosis of Ewing's sarcoma is improving with radiotherapy and chemotherapy. Further cases are needed to study the biological behaviour of primary cranial Ewing's sarcoma.