Initial Radical Resection Research Articles

Case report of recurrent fibromatosis with laryngeal involvement: Treatment based on network analyses of NGS data.

Aggressive fibromatosis (AF) is a rare, benign neoplasm originating from musculoaponeurotic stromal structures characterized by aggressive growth and infiltration of local tissues. To date, only six previous cases of AF involving the larynx have been reported. The present case was that of a 70-year-old female patient with a 5-year history of hoarseness and an enlarging neck mass consistent with aggressive recurrent fibromatosis. MRI displayed a large, solid mass arising from the left anterior cervical space displacing the trachea and upper airway to the right. At one year after the initial radical resection, the patient presented with recurrence. A second radical excision was performed not including a laryngectomy. Radiation therapy was considered for possible local treatment to prevent subsequent tumor recurrence. According to the literature, AF has recurrence rates of up to 40-70% within 18 months. There is only sparse literature to guide treatment. Using the mutations detected in the patient's AF tissue, an Ingenuity Pathway Analysis (IPA) was used to guide treatment of the recurrence. In the present case, the IPA analysis indicated the use of pazopanib to treat the patient's cancer. In general, surgery appears to be the treatment of choice for head and neck AF, but the management of recurrence is controversial.

Recurrent Aggressive Fibromatosis with Laryngeal Involvement: A Case Report and Literature Review

Aggressive Fibromatosis (AF) is a rare, benign neoplasm originating from musculoaponeurotic stromal structures characterized by aggressive growth and infiltration of local tissues. To date, there are only six previously reported cases of AF involving the larynx. Our case involves a 70-year-old female with a 5-year history of hoarseness and an enlarging neck mass consistent with aggressive recurrent fibromatosis. MRI showed a large, solid mass arising from the left anterior cervical space displacing the trachea and upper airway to the right. A year after the initial radical resection, the patient presented with recurrence. A second radical excision was performed not including a laryngectomy. Radiation therapy was consulted for possible local treatment to prevent subsequent recurrence of tumor. Literature has reported up to 40-70% recurrence rates of AF within 18 months. There is minimal literature to guide treatment. Surgery seems to be the treatment of choice for head and neck AF, but management of recurrence is controversial.

Near-Total Laryngectomy for Extranodal Infiltration from Papillary Cancer to Larynx and Hypopharynx

Papillary thyroid cancer usually presents with limited loco-regional disease. Rarely extrathyroidal extension to the surrounding vital structures is seen. It is rarer for metastatic node from papillary thyroid cancer to directly infiltrate into the larynx or pharynx. We report a rare case where an extranodal extension from a metastatic papillary thyroid cancer infiltrated into the larynx and pyriform sinus in a 56-year-old female. Near-total laryngectomy with partial pharyngectomy was done for gross removal of the disease to achieve long-term locoregional disease-free survival. Although locally advanced thyroid cancer is rare, once noted, it presents challenge to the surgeon to achieve gross total removal of the tumor. Initial radical resection should be done to achieve gross total removal of the cancer.

Surgical treatment of recurring ameloblastoma, are there options?

Our aim was to evaluate the treatment given to patients with intraosseus ameloblastomas with special emphasis on recurrence and the outcomes of primary and secondary resection. Forty-eight patients who were treated for intraosseous ameloblastoma at 8 centres across Sweden met the inclusion criteria. They showed typical distribution of age, sex, site of lesion, and characteristic presenting features. Eleven of the 48 were initially treated with radical resection and none recurred. Twenty-two of the remaining 37 who were initially treated by conservative resection presented with recurrences. Sixteen of the 22 then had conservative secondary resections, which resulted in further recurrence in 6 patients. Initial radical resection is therefore superior to conservative management as far as recurrences are concerned. We argue, however, that a conservative surgical approach is adequate for many intraosseous ameloblastomas with limited extension, because relapse can be followed by radical resection if clinically indicated in selected cases.

The Role of Surgery in the Management of Recurrent Adrenocortical Carcinoma

Surgery is the standard of care for localized adrenocortical carcinomas, but its role for recurrent disease is not well defined. Our objective was to evaluate clinical outcome after surgery for recurrence. We conducted a retrospective analysis in 154 patients with first recurrence after initial radical resection from the German Adrenocortical Carcinoma Registry. We evaluated progression-free survival (PFS) and overall survival (OS) by Kaplan-Meier method and identified prognostic factors by Cox regression analysis. A total of 101 patients underwent repeated surgery (radical resection, n = 78), and 99 received (additional) nonsurgical therapy. After a median of 6 (1-221) months, 144 patients (94%) experienced progression. Multivariate analysis adjusted for age, sex, tumor burden, time to first recurrence (TTFR), surgery for recurrence (including resection status), and additional therapy indicated that only two factors were significantly associated with shorter PFS [hazard ratio for progression: for TTFR ≤ 12 months, 1.8 (95% confidence interval = 1.3-2.6) vs. TTFR > 12 months; for macroscopically incomplete resection, 3.4 (1.5-7.9), and for no surgery, 3.4 (1.6-7.0) vs. microscopically complete (R0)-resection and OS [hazard ratio for death: for TTFR > 12 months, 3.1 (2.0-4.7) vs. TTFR ≤ 12 months; for macroscopically incomplete resection, 2.7 (1.1-6.9), and no surgery, 4.2 (1.8-9.6) vs. R0-resection]. Patients who had both TTFR over 12 months and R0-resection of recurrent tumors (n = 22) had the best prognosis (median PFS, 24 months; median OS, >60 months). The best predictors of prolonged survival after first recurrence are TTFR over 12 months and R0-resection. Our data suggest that patients with longer TTFR and tumors amenable to radical resection should be operated, whereas individualized treatment decisions are needed for patients with short TTFR or with not completely resectable tumors.

Hydrocephalus with brain tumors in children

Tumor-associated hydrocephalus is common in primary pediatric brain tumors. The managements involve radical tumor resection, temporary external ventricular drainage, and different definite shunting procedures. The purpose of this study is to sum up our experience of definite shunting procedures for tumoral hydrocephalus in children and correlate with reported literatures. This is a retrospective review of a series of 1,250 cases of primary pediatric brain tumors in patients <18years of age collected in Taipei Veterans General Hospital from 1971 to 2008. Cases with questionable records about hydrocephalus were excluded. A total of 56.7% of cases presented hydrocephalus, including hydrocephalus that occurred at tumor diagnosis (51.5%), and hydrocephalus developed after tumor diagnosis (5.1%). At tumor diagnosis, the hydrocephalus was mainly obstructive type (98%) and rarely communicating type (1.9%). Definite shunting procedures in this series comprised of ventriculoperitoneal (VP) shunt in 54.4%, endoscopic third ventriculostomy (ETV) in 10.9%, subduroperitoneal (SP) shunt in 4.8%, septostomy in 0.7%, lumboperitoneal shunt in 0.6%, and ventriculoatrial shunt in 0.1% of patients with hydrocephalus. There was a tendency of decreasing requirement and changing timing for VP shunt implantation. There was a gradual increase in usage of ETV for hydrocephalus in specific types and locations of tumors. In the past two decades, we tended to use the VP shunt more cautiously for obstructive tumoral hydrocephalus. We try to perform initial radical resection of tumors as indicated and the more frequent use of ETV in selective cases that help to decrease the requirement of VP shunt implantation.

A case of intravascular epithelioid hemangioendothelioma occurring 14 years after coil embolization for an extracranial internal carotid artery aneurysm

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm originating from various organs. The clinical outcome mostly depends on surgical resectability. The authors report an EHE of the extracranial internal carotid artery developed in a 59-year-old male patient 14 years after the intravascular coil embolization for a carotid aneurysm at the same site. Because the lesion was initially diagnosed as regrowth of the thrombosed aneurysm, decision for radical resection was delayed, and the patient died from rapid tumor progression. Differential diagnosis of atypical vascular mass lesions should include neoplasm, because initial radical resection may be the key to achieve a better prognosis.

Recurrent epithelioid sarcoma in the thoracic spine successfully treated with multilevel total en bloc spondylectomy

Epithelioid sarcoma (ES) is a rare type of soft tissue tumor. The common location of ES is at the extremities and rarely occurs in axial skeleton. Only two cases have been reported so far. Initial wide resection is recommended for the treatment of ES. However, the local recurrent rate is high and repeat surgical resection is still an option for the treatment of the recurrent. In the spine, however, the proper treatment of recurrent ES has not yet been published. Therefore, the objective of this case report is to illustrate the management strategies for the local recurrent ES after initial surgical resection in the thoracic spine. A 14-year-old boy was diagnosed for ES in the thoracic spine for 2years. He was first treated by surgical resection followed by the chemotherapy and radiotherapy but the disease had progressed and the spine was gradually deformed. He was admitted to our facility with a large soft tissue mass, severe kyphotic deformity and neurological deficit. We removed the tumor en bloc by one-stage posterior only approach. The posterior transpedicular spinal instrumentation and fibular strut graft were used for the reconstruction. On the last follow-up, 2year after the surgery, the patient remained in good condition. In conclusion, the recurrent ES of the spine can still archive a good oncological outcome with repeat radical resection, but the initial radical resection remains the best treatment option in order to retard the relentless course of this kind of malignancy.

Epithelioid sarcoma in the thoracic spine

Epithelioid sarcoma is a rare and highly malignant soft tissue tumor that is commonly found in the extremities and rarely in the trunk area. This malignant tumor often mimics granuloma or nodular fasciitis, which causes a delay in establishing the diagnosis. This type of cancer has a high recurrence rate. Surgical treatment requires wide radical resection. The objective of this case report is to highlight the unique location of a rare neoplasm and to illustrate the relentless course of epithelioid sarcoma despite initial radical resection. A 14-year-old boy was admitted to our facility with a soft tissue mass on the right lower thoracic spine. The large tumor mass had deeply penetrated into the muscles, infiltrated the neuroforamen of T9–T10 level, and compressed the dural sac. Immunohistological study of the biopsy was highly consistent with an epithelioid sarcoma. Wide excision of the mass, laminectomy and spine fusion with instrumentation was performed. The patient received chemotherapy and irradiation. The first recurrence of the neoplasm was seen as a contralateral metastasis 21 months after the resection. On the last follow-up, 3 years postoperatively, the patient was in a good general condition. However, further progression of the sarcoma had to be recognized. Our case encompasses multiple features that represent negative prognostic factors. Initial wide excision of the neoplasm and adjuvant therapy including chemotherapy and irradiation seem to slow down the relentless course of epithelioid sarcoma in the trunk.