BNCT for primary synovial sarcoma.

Synovial sarcoma is a rare tumor requiring new treatment methods. A 46-year-old woman with primary monophasic synovial sarcoma in the left thigh involving the sciatic nerve, declining surgery because of potential dysfunction of the affected limbs, received two courses of BNCT. The tumor thus reduced was completely resected with no subsequent recurrence. The patient is now able to walk unassisted, and no local recurrence has been observed, demonstrating the applicability of BNCT as adjuvant therapy for synovial sarcoma. Further study and analysis with more experience accumulation are needed to confirm the real impact of BNCT efficacy for its application to synovial sarcoma.

Excision of recurrent synovial sarcoma of the infratemporal fossa.

Synovial sarcoma is a soft tissue sarcoma especially encountered in the lower extremities. The infratemporal fossa is quite a rare location. Since it is a closed location, combined approaches and multidisciplinary planning always need to be considered. This case emphasizes the high-grade character of synovial sarcoma, which causes it to recur often. The difficulty of clear surgical margins in the infratemporal fossa adds to synovial sarcoma a second challenging issue. Therefore, the need of complementary therapy is essential. In this case, we applied postoperative radiotherapy and we did not observe any sign of local, regional or distant metastasis in the one-year follow-up. In this article, we present a 68-year-old male patient together with totally excised synovial sarcoma in the infratemporal fossa by mandibular swing and transzygomatic technique information on the manifestation, imaging, histopathological features and postoperative complications of previous infratemporal fossa synovial sarcomas.

Modified technique of functional vertical hemilaryngectomy for cancer invading 1 hemicricoid.

BACKGROUND: Partial laryngectomy techniques are challenging, especially in young patients where function has to be preserved without compromising the oncologic outcome. We present a modified laryngectomy technique indicated for tumors invading one hemicricoid. METHODS: Vertical hemilaryngectomy with reconstruction of neo-glottis by hemi trachea and placement of an endolaryngeal silicon prosthesis for a 21-y old female patient presenting with a synovial sarcoma located on the left arytenoid area. RESULTS: The prosthesis was removed at 6 w with tracheotomy closure at 8w post-op. Patient underwent adjuvant radiotherapy. Functional outcome showed good swallowing without aspiration. Voice was hoarse. At 5 years post-op the patient is free of recurrence presenting only mild dyspnea upon effort. CONCLUSION: Vertical hemilaryngectomy including a hemicricoid is feasible with single stage reconstruction by a hemi-trachea of 4 to 5 rings intussuscepted into the thyroid cartilage. The functional outcome is good considering oncologic safety and avoidance of a permanent tracheostomy. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1708-1716, 2016.

Aortic homograft reconstruction of partial laryngectomy defects: a new technique.

OBJECTIVES: Wide-field transcervical partial laryngectomy often precludes tracheotomy decannulation. It is done infrequently today, primarily because of the popularity of chemotherapy-radiotherapy treatment regimens and limited enthusiasm for using transcervical partial laryngectomy after failed radiotherapy. We sought to identify a new reconstructive technique that would provide an alternative to total laryngectomy in as many patients as possible. METHODS: We performed a retrospective examination of 15 patients who underwent single-stage wide-field transcervical partial laryngectomy with cryopreserved aortic homograft reconstruction. Eight of the 15 patients had previously undergone failed radiotherapy. At least 40% of the cricoid circumference was resected in 8 patients. RESULTS: All 15 patients had their tracheotomy tube removed and have laryngeal phonation, and 14 of the 15 resumed oral intake. There were no major surgical complications. CONCLUSIONS: Use of aortic homografts is a new, reliable, and versatile reconstructive option for performing conservation laryngeal cancer surgery that allows for airway, swallowing, and voice preservation.

Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension.

BACKGROUND: The medical literature on synovial sarcoma (SS) of the head and neck region is limited. Thus, we determined whether clinical characteristics and treatment were associated with recurrence and survival rates in patients with SS of the head and neck. METHODS: We retrospectively identified patients with a pathologic diagnosis of SS of the head and neck at our institution (a large tertiary comprehensive cancer center) and compared recurrence and survival rates by clinical characteristics and treatment. RESULTS: Forty patients with SS of the head and neck were identified from 1945 to 2004 (first case in 1968), representing <5% of all head and neck sarcomas seen at our institution during this time period. Twenty-three patients (58%) had the monophasic histologic subtype, 15 (38%) biphasic, and 2 unspecified. Most patients were male (73%), with a median age of 29 years. SS tumors were most commonly located in the neck (60%); thus, the most common symptoms were a neck mass and neck pain. No patients reported a history of radiation exposure. Higher disease-specific and overall survival rates were associated with upper aerodigestive tract location, tumors of < or =5 cm, and tumors did not extend into bone. Patients treated with surgery and adjuvant radiotherapy had higher survival and lower recurrence rates than did those treated with surgery alone or a combination of surgery, radiotherapy, and chemotherapy. This difference was not significant, and the subgroups were small, with substantial confounding by adverse prognostic factors. CONCLUSIONS: SS of the head and neck is extremely rare, and our results should be viewed with caution given the relatively small group size and treatment over a 36-year period. Survival rates were associated with tumor location, size, and extension. Treatment of SS of the head and neck should be directed toward complete surgical resection. Given the known sensitivity of SS to contemporary chemotherapy, a multimodality approach should be considered in the perioperative setting, especially in high risk patients.

Clinical outcome of a novel photodynamic therapy technique using acridine orange for synovial sarcomas.

Synovial sarcoma (SS) is one of common malignant soft-tissue tumors and is encountered most commonly in children and young adults. It frequently involves or invades major neurovascular structures and bones, and its local recurrence rate after simple resection has been reported to be as high as up to 80%. Because major nerves and vessels, as well as an adequate amount of bone, must be preserved to restore excellent limb function in cases of SS, a surgical technique entailing a low risk of local recurrence is needed. Based on the findings of recent experimental studies conducted by us using a mouse osteosarcoma model, we developed a novel therapeutic technique for SS, consisting of reduction surgery followed by photodynamic therapy using acridine orange (AO-PDT), with or without X-ray irradiation at 5 Gy. A preliminary study revealed that low-dose X-rays also excite AO like photons. After an initial study on cell cultures, this novel technique was applied to six cases of SS. A follow-up of the subjects to determine the clinical outcome revealed that none of the cases treated by AO-PDT, including the four cases treated by additional 5 Gy irradiation and the two cases not receiving any radiation, showed any evidence of recurrence or local/systemic complications during the follow-up period of 19-51 months after the surgery. Therefore, we believe that AO-PDT with 5 Gy irradiation may be an excellent novel therapeutic modality with reduction surgery to salvage excellent limb function in SS involving major nerves and vessels or bones.

Ukrain treatment of a patient with retroperitoneal synovial sarcoma. Case report.

A 23-year-old woman, diagnosed with a synovial sarcoma of the peritoneum, underwent an operation for tumor extraction. In the postoperative period, Ukrain was injected i.v. at a dose of 10 mg on alternate days, for a total of 10 injections. After a 2-month break, this schedule was repeated. Ukrain treatment was well tolerated by the patient and there were no complications in the postoperative period. The following changes in immunohematological parameters were observed: increased total leucocytes, T lymphocytes and T helpers. Nearly 4 years after Ukrain therapy, the patient is in complete remission.

Tumor-induced hypercalcemia in a patient with extensive soft tissue sarcoma: effects of bisphosphonate therapy and surgery.

Tumor-induced hypercalcemia (TIH) is a frequent complication of advanced cancer, but it has been rarely reported in patients with sarcoma. We describe the case of a young female patient with TIH and with an extensive synoviosarcoma of the left lower limb destroying the bony structures. Hypercalcemia was severe (18.3 mg/dl) and accompanied by low serum Pi and suppressed parathyroid hormone (PTH) and 1,25(OH)2 vit D3 serum concentrations. Hypercalcemia was successfully treated with ibandronate, a new third-generation bisphosphonate, and radical surgery was performed when the patient was normocalcemic. Circulating levels of PTH-related protein (PTHrP) were elevated at 22.5 pmol/L (NI < 9). PTHrP levels did not change after successful therapy of TIH, in contrast with PTH, which increased sharply. PTHrP levels were normalized after radical surgery. Moreover, low serum Pi with reduced threshold for phosphate excretion and increased tubular calcium reabsorption supported the notion that PTHrP was indeed the essential mediator of paraneoplastic hypercalcemia in this case despite the extensive bone destruction.

Tetany due to hypomagnesemia induced by cisplatin and doxorubicin treatment for synovial sarcoma.

Hypocalcemic crisis developed in a patient with monophasic synovial sarcoma after amputation of the right leg, followed by long-term treatment with cisplatin and doxorubicin. Laboratory data revealed severe hypomagnesemia and hypocalcemia. High normal intact parathyroid hormone (PTH), elevated mid-region PTH and undetectable osteocalcin levels had already been found before the appearance of obvious symptoms concomitantly associated with moderate hypomagnesemia and hypocalcemia. Further, both PTH levels measured by two different methods gradually decreased until the initiation of magnesium supplementation. The magnesium supplement immediately relieved the tetany, and induced striking increases in both intact and mid-region PTH levels transiently and continuous elevations of osteocalcin levels. These results suggest that magnesium depletion has dual effects on PTH secretion, from stimulation to inhibition, as hypomagnesemia progresses. Both relative hypoparathyroidism and refractoriness of bone to PTH were thought to be responsible for hypocalcemia due to hypomagnesemia.