Primary Management of Squamous Cell Carcinoma of the Anal Canal: A 30-year Community Hospital Experience.

We reviewed outcomes of 41 patients treated with curative-intent radiotherapy for anal canal carcinoma at a community hospital between 1985 and 2015. Twenty-six (63%) presented with stage I or II disease while 15 (37%) had stage III. Thirty-seven received definitive chemoradiation and 4 radiotherapy alone. Thirteen (31.7%) received ≤59.4Gy. Thirty-two (78%) were treated with 3-dimensional conformal radiotherapy while 9 (22%) received intensity-modulated radiotherapy. At 5 years, local control, regional control, freedom from distant metastasis, cause-specific survival, and overall survival were 80%, 98%, 88%, 77%, and 51%. Of those who received >59.4Gy, local control and overall survival were not improved.

Radiotherapy Alone or With Chemotherapy in the Management of Carcinoma of the Supraglottic Larynx: A 25-Year Community Hospital Experience.

OBJECTIVES: The purpose of this study was to retrospectively review outcomes for patients treated with definitive radiotherapy for carcinoma of the supraglottic larynx at a community hospital and to compare our results with the literature. MATERIALS AND METHODS: Treatment records of 46 patients with localized carcinoma of the supraglottic larynx treated from January 1987 through January 2012 were reviewed. Overall, 18 (39%) presented with stage I or II disease, whereas 28 (61%) presented with stage III to IV. In total, 30 patients (65%) were treated using hyperfractionation, whereas 16 (35%) received once-daily fractionation. Twelve patients (26%) received concurrent chemotherapy with weekly cisplatin. The Kaplan-Meier method was used to assess outcomes. RESULTS: The median follow-up for the living patients was 6.7 years (range, 1.7 to 23.1 y). At 5 years, the local-regional control (LRC), ultimate LRC, laryngeal preservation, and overall survival (OS) rates were 70%, 82%, 65%, and 53%, respectively. At 5 years, disease-free survival and cause-specific survival rates were 75% and 76%, respectively. The 5-year OS rates by American Joint Committee on Cancer stage were as follows: I to II, 61%; III, 51%; and IV, 44%. For those receiving concurrent chemotherapy, there was no improvement in 5-year LRC (83% vs. 66%; P=0.4081) or OS (55% vs. 50%; P=0.7697). For those receiving hyperfractionation, there was no improvement in 5-year LRC (75% vs. 63%; P=0.3369) or OS (55% vs. 50%; P=0.4161). CONCLUSIONS: Our outcomes are similar to those reported in the literature. Neither hyperfractionation nor chemotherapy appeared to confer a benefit for disease control or OS possibly owing to small sample size and the inherent bias of a retrospective review.

Merkel cell carcinoma.

PURPOSE: To determine the natural history and treatment outcomes for patients with Merkel cell carcinoma. METHODS: Review of the literature. RESULTS: The probability of regional node involvement at presentation exceeds 50%; few patients present with distant metastases. Comprehensive treatment of the primary site and regional lymphatics with surgery or radiotherapy results in the highest likelihood of cure. The role of adjuvant chemotherapy remains investigational. CONCLUSION: The probability of regional dissemination at diagnosis is high. The optimal treatment is resection of the primary tumor and treatment of the regional lymphatics. Resection of all gross tumor should be accomplished followed by local-regional radiotherapy in most patients.

Submandibular gland-sparing intensity-modulated radiotherapy.

Xerostomia is one of the most debilitating late effects of head and neck radiotherapy (RT) and significantly impacts quality of life. The submandibular gland (SMG) provides approximately 70% of the unstimulated saliva that accounts for about 95% of the salivary flow during a 24-hour period. Intensity-modulated RT (IMRT) has been used in recent years to lower the RT dose to the parotid gland(s) to reduce long-term xerostomia. There is little experience with SMG-sparing IMRT. Mean RT doses to the SMG exceeding 39 Gy cause permanent ablation of both stimulated and unstimulated salivary flow. Limited data suggest that SMG-sparing IMRT in selected patients results in reduced long-term xerostomia without increasing the risk of a local-regional recurrence.

Radiotherapy alone or combined with chemotherapy for the treatment of squamous cell carcinoma of the base of the tongue.

OBJECTIVES: To evaluate the efficacy of definitive radiotherapy (RT) for the treatment of base of the tongue cancer in a private practice. METHODS: We retrospectively reviewed the medical records of 48 patients with squamous cell carcinoma of the base of the tongue treated with definitive RT and followed from 0.2 to 15.2 years (median, 2.7 y). Follow-up of living patients ranged from 1.5 to 15.2 years (median, 4.9 y). Two thirds of the patients received twice-daily treatment. The median total dose was 74.4 Gy (range, 65 to 76.8 Gy). The median overall treatment time was 45.5 days (range, 36 to 68 d). There were 3 patients (6%) with clinically positive neck nodes who received a neck dissection. Adjuvant chemotherapy was given to 28 patients (58%) with advanced local-regional disease; 1 patient (4%) received induction chemotherapy and 27 patients (96%) received concomitant chemotherapy. Toxicity was graded according to the Common Terminology Criteria for Adverse Events, v3.0. RESULTS: Local control rates by T stage at 5 years were as follows: T1 to T2, 91%; T3, 83%; and T4, 12%. The 5-year rates of local-regional control were as follows: I to II 80%; III, 89%; IVA, 59%; and IVB, 42%. The rates of overall survival at 5 years were as follows: I to II, 67%; III, 56%; IVA, 45%; and IVB, 33%. The 5-year rates of cause-specific survival were as follows: I to II, 67%; III, 76%; IVA, 50%; and IVB, 60%. Seven patients (15%) experienced severe late complications. CONCLUSIONS: Our data reveal that the local-regional control and overall survival rates after definitive RT were comparable with those in the literature. Twice-daily radiation is well tolerated with moderate-late toxicity, which is consistent with the observations of others.

Head and neck paragangliomas.

BACKGROUND: The purpose of this study was to describe the natural history and optimal treatment for head and neck paragangliomas (PGs). METHODS: Our methods were the review of the pertinent literature. RESULTS: PGs are rare tumors seen most commonly in the head and neck. Approximately 90% are sporadic; the remainder are familial and related to mutations of the succinate dehydrogenase (SDH) gene complex. Most PGs are benign and slow growing; 6% to 19% are malignant, as evidenced by the development of metastases. PGs may be treated by complete resection or moderate-dose radiotherapy with a ≥90% likelihood of cure. The optimal radiotherapy dose is approximately 45 Gy/25 fractions/5 weeks. The treatment modality selected depends on the risk of complications. Due to their rarity, the optimal treatment for malignant PGs is unclear. CONCLUSION: PGs may be treated by either complete resection or radiotherapy with a high likelihood of success. Treatment depends on the location and extent of the PG and the morbidity associated with treatment.

Radiation therapy for management of t1-t2 glottic cancer at a private practice.

PURPOSE: The purpose of this study was to retrospectively analyze patients with T1-T2N0 squamous cell carcinomas of the glottic larynx treated at a private institution, to review the pertinent literature, and to compare our outcomes to those of academic institutions. METHODS AND MATERIALS: A total of 118 patients were treated with radiation therapy between May 1987 and August 2006 at a private institution and followed up for ≥2 years. Three patients were lost to follow-up between 18 and 19 months. RESULTS: The 5-year local control rates were: T1a, 91%; T1b, 95%; T2a, 96%; and T2b, 100%. The 5-year ultimate local control rates after irradiation and including patients who were successfully salvaged with surgery after a local recurrence were: T1a, 94%; T1b, 100%; T2a, 96%; and T2b, 100%. Eight (7%) of the 118 patients developed a local recurrence. There were no isolated regional or distant recurrences. The 5-year overall survival rates were: T1a, 73%; T1b, 78%; T2a, 62%; and T2b, 69%. The 5-year cause-specific survival rates were: T1a, 96%; T1b, 100%; T2a, 100%; and T2b, 100%. Two patients experienced severe complications. CONCLUSION: Patients with limited T1aN0 cancers may be treated with either transoral laser excision or RT. Those with more advanced T1-T2N0 cancers are treated with definitive RT. We do not advocate elective nodal irradiation, even for those with bulky T2B malignancies. The addition of concomitant weekly cisplatin 30 mg/M² is considered for patients with T2B cancers. Open parotid laryngectomy is reserved for salvage of suitable patients with a local recurrence.

The management of adult soft tissue sarcomas.

Soft tissue sarcomas are a relatively rare, heterogeneous group of tumors arising from mesenchymal tissues and occurring almost anywhere in the body. The rate of progression and likelihood of hematogenous dissemination, usually to the lung, is determined primarily by tumor grade. The likelihood of regional spread is low. Pretreatment evaluation includes computed tomography of the primary site and chest and magnetic resonance imaging of the primary tumor. The mainstay of treatment is surgery; wide excision for low-grade lesions and wide or radical (compartmental) resection for high-grade tumors. Often, these procedures cannot be achieved either because of the location and extent of the sarcoma or anticipated functional deficit. Adjuvant preoperative and/or postoperative radiotherapy improves the likelihood of local control and preserves function when adequate margins cannot be achieved with surgery alone. The role of adjuvant chemotherapy is unclear; however, some data suggest the doxorubicin containing regimens may improve the likelihood of cure for high-grade lesions, particularly large tumors arising in the extremities. Prognosis is influenced by a variety of factors, including age, tumor size, histologic grade, depth (superficial or deep), histologic subtype, and site. Approximately 90% and 98% of recurrences are observed within 5 years and 10 years, respectively. Five-year overall survival rates range from approximately 60% to 80%.

Radiotherapy in the treatment of resectable rectal adenocarcinoma.

The goal of treatment is to cure whereas maintaining sphincter function and minimizing toxicity. Although the mainstay of the treatment is surgery, radiotherapy (RT) is used in a substantial proportion of patients depending on the location and extent of the tumor. The aim of this article is to discuss the role of RT in patients with resectable rectal adenocarcinoma. This article is a review of the pertinent literature. Results show that patients with T1N0 exophytic, well to moderately differentiated, mobile tumors < or = 3 cm in diameter may be treated with either transanal excision or endocavitary RT. The probability of cure with either approach is approximately 80% to 90% and depends on selection criteria. The advantages of endocavitary RT are that it is an outpatient procedure requiring, at most, local anesthesia and is suitable for elderly, infirm patients. The disadvantage is that few of these treatment units are available. Patients who experience a local-regional recurrence may be surgically salvaged. Patients who undergo transanal excision and have unfavorable pathologic findings including equivocal or close margins, poor differentiation, invasion of the muscularis propria, and/or endothelial-lined space invasion have a high risk of local-regional recurrence after surgery alone. The addition of postoperative RT improves the likelihood of cure from 85% to 90%. Patients presenting with unfavorable tumors that are borderline resectable with a transanal excision may be downstaged with preoperative RT and rendered suitable for a wide local excision. The addition of concomitant chemotherapy probably enhances downstaging and may improve the likelihood of sphincter preservation. Patients with T3 and/or N1 rectal cancers have a relatively high probability of local-regional recurrence after surgery alone. Preoperative RT and postoperative RT combined with adjuvant chemotherapy have been shown to significantly reduce the risk of local-regional recurrence and improve survival. Whether preoperative RT alone or combined with chemotherapy is more efficacious than postoperative chemoradiation remains unclear. Endocavitary RT or transanal excision is suitable for patients with T1N0 cancers. Depending on tumor location and extent, adjuvant RT may improve the probability of local-regional control and survival for patients with locally advanced rectal adenocarcinomas.

Salivary gland pleomorphic adenoma.

We discuss the optimal treatment and outcomes for pleomorphic adenoma of the salivary glands by reviewing the pertinent literature. Pleomorphic adenoma is the most common benign salivary gland neoplasm. It is found mostly in the parotid gland in middle-aged women. It progresses slowly and, left untreated, can produce significant morbidity and, rarely, death. The optimal treatment is superficial or total parotidectomy with facial nerve preservation, which results in local control rates of 95% or higher. Radiotherapy (RT) is useful to obtain local control in patients with positive margins, unresectable tumors, and multifocal recurrences after prior resection. Local control rates after RT for microscopic and gross residual tumor are approximately 80% to 85% and 40% to 60%, respectively. The main complication is surgically induced 7th nerve injury. Surgery is the mainstay of treatment and results in a very high cure rate. RT increases the likelihood of local control in the small subset of patients with incompletely resectable tumors and/or multifocal recurrences.

Re-irradiation of head and neck carcinoma.

To determine the efficacy of re-irradiation of patients with head and neck carcinoma we reviewed the pertinent literature. Depending on the location and extent of the tumor, re-irradiation may be accomplished with external beam radiotherapy (RT), brachytherapy, intraoperative RT, and/or radiosurgery. The likelihood of cure is impacted by the interval between the initial course of RT and re-irradiation, whether the carcinoma is a recurrence or a second primary tumor, initial T-stage, and recurrent T-stage (rT-stage), whether the gross disease is isolated or local-regional, and histology. Patients with T1-T2 N0 second primary tumors have a relatively high likelihood of cure, whereas those with recurrent T3-T4 cancers, a short disease-free interval, and/or associated with a regional recurrence have a very low probability of cure. The likelihood of severe late complications is relatively high and is related to prior RT dose, primary site, retreatment RT dose, treatment volume, and technique. Previously irradiated patients with T1-T2 N0 carcinomas who are not surgical candidates may benefit from re-irradiation, albeit with an elevated risk of late complications. Those with advanced recurrences are unlikely to benefit from re-irradiation and should be considered for either a prospective clinical trial or for palliative chemotherapy or supportive care.

Cutaneous angiosarcoma.

OBJECTIVE: To discuss the treatment and outcomes for cutaneous angiosarcoma. METHODS: Review of the pertinent literature. RESULTS: Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. It usually arises in the scalp or face and is locally advanced at presentation. Patients are most often white, male, and elderly. A subset of patients presents with multifocal disease and/or positive regional nodes. Although the optimal treatment is surgery followed by wide-field radiotherapy (RT), the disease is frequently so extensive at diagnosis that it is not completely resectable. Even after optimal local-regional treatment, there is a relatively high likelihood of a local recurrence at the margins of the RT fields. The probability of hematogenous dissemination is relatively high. Limited data suggest that chemotherapy may be useful for palliation with progression-free survival rates ranging from 1 to 5 months. The 5-year local-regional control rates are approximately 40% to 50%, the 5-year distant metastasis-free survival rates range from 20% to 40%, and the 5-year survival rates range from 10% to 30%. CONCLUSION: Cutaneous angiosarcoma is a rare, aggressive malignancy that is optimally treated with resection and wide-field postoperative RT. The likelihood of local-regional failure is high, as is the risk of distant relapse. Chemotherapy may be useful for short-term palliation.

Sinonasal undifferentiated carcinoma.

PURPOSE: The purpose of this paper is to discuss the treatment and outcomes for patients with sinonasal undifferentiated carcinoma. METHODS: Review of the pertinent literature. RESULTS: Most series contain a limited number of patients treated with various combinations of surgery, radiotherapy (RT), and chemotherapy. Follow-up periods for disease-free patients are sometimes relatively short. The majority of patients present with locally advanced tumors; 10% to 30% have clinically positive regional nodes. The risk of local-regional recurrence after treatment is probably 20% to 30% or higher, depending on the extent of disease. The likelihood of distant dissemination is approximately 25% to 30% and the cure rate varies from roughly 20% to 50%. Better outcomes are observed in patients treated with craniofacial resection combined with pre- or postoperative RT alone or with adjuvant chemotherapy. This is probably due, in part, to selection bias. Patients with incompletely resectable tumors are best treated with definitive RT and adjuvant chemotherapy. CONCLUSION: The optimal treatment is craniofacial resection combined with adjuvant RT alone or with chemotherapy. The risk of relapse is relatively high and the probability of cure is modest. Patients with incompletely resectable tumors may sometimes be cured with definitive chemoradiation.

Pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVNS) is a rare proliferative disorder that affects the synovium in young and middle-aged adults. Although most believe that it is an inflammatory process, some believe that it is a benign neoplasm. The optimal treatment is surgery. The local recurrence rate after marginal excision for localized PVNS is low. In contrast, the local recurrence rate after open synovectomy for diffuse PVNS is relatively high. The intra-articular instillation of radioactive isotopes or external beam radiotherapy (approximately 35 Gy in 14-15 fractions over 3 weeks) may significantly improve the likelihood of local control and long-term function in patients with incompletely resected diffuse PVNS. The probability of complications after moderate-dose radiotherapy (RT) is low.

Adult head and neck soft tissue sarcomas.

BACKGROUND: The purpose was to determine the optimal treatment for adult patients with head and neck soft tissue sarcomas. METHODS: We conducted a review of the pertinent literature. RESULTS: Local control after surgery alone or combined with radiotherapy was obtained in approximately 60% to 70% of the patients. The probability of local control is influenced by histologic grade, tumor size, and surgical margins. Patients with high-grade tumors and/or positive margins have improved local control if adjuvant radiotherapy is used. Distant metastases occurred in 10% to 30% of patients. The 5-year overall and cause-specific survival rates varied from approximately 60% to 70% and are affected by age, histologic grade, previous treatment of tumor, invasion of deep structures, and adequacy of surgery. CONCLUSIONS: The optimal treatment for adult head and neck soft tissue sarcomas is surgery. Adjuvant radiotherapy improves outcomes for those with high-grade tumors and/or positive margins. Radiotherapy alone will cure a small subset of patients with unresectable tumors.

Skull base chordoma.

PURPOSE: Our purpose was to discuss the optimal treatment and outcomes for patients with skull base chordomas. METHODS: We reviewed the pertinent literature for this study. RESULTS: Skull base chordomas usually arise in the clivus and are rarely completely resectable. Therefore, most are treated with radiotherapy (RT). Because of the risk of severe late complications, the dose is often limited with conventional photon RT, and the probability of cure is low. Proton RT alone or combined with photon RT (proton/photon RT) offers the advantage of improved dose distribution and the ability to treat the tumor to a higher dose without exceeding normal tissue tolerance. The 10-year local control rate after proton/photon RT is approximately 40% to 50%. The probability of local control is related to minimum tumor dose and dose inhomogeneity. CONCLUSIONS: Skull base chordoma is a rare neoplasm that is rarely cured after surgery alone or combined with conventional RT. Proton/photon RT offers the advantage of increasing the tumor dose while minimizing the dose to normal tissues, thus reducing the risk of late complications. The optimal treatment may be photon/proton RT alone or combined with a gross total resection, when feasible.

Merkel cell carcinoma: treatment and outcomes.

BACKGROUND: The purpose of the current study was to determine the outcomes of patients with previously untreated Merkel cell carcinoma of the skin who were treated with curative intent. METHODS: Between October 1984 and January 2002, 34 patients were treated with radiotherapy alone (2 patients) or combined with surgery (32 patients). Nine patients received adjuvant chemotherapy. Patients had follow-up for a median of 3.0 years (range, 0.3 to 18.5 yrs). Follow-up on living patients ranged from 2.2 to 18.5 years (median, 7.1 yrs). RESULTS: The 5-year outcomes were as follows: local control, 94%, locoregional control, 80%; freedom from distant metastases, 60%; cause-specific survival, 52%; and survival, 37%. No patient experienced a severe complication. CONCLUSIONS: Patients treated aggressively with surgery and locoregional radiotherapy have about a 50% chance of cure. Limited data suggest that definitive radiotherapy alone or after incomplete macroscopic resection may control locoregional disease in a significant subset of patients. The dominant site of failure was distant.

Solitary plasmacytoma of bone and soft tissues.

PURPOSE: To define the optimal treatment and outcomes for patients with solitary plasmacytoma of bone and soft tissue. METHODS: Review of the literature. RESULTS: Solitary plasmacytomas are uncommon and account for less than 5% of plasma cell neoplasms. Solitary plasmacytomas of bone (SPB) usually occur in the vertebra and skull and are more common than extramedullary plasmacytomas (EMP) that almost always arise in the head and neck and may spread to regional lymph nodes. The optimal treatment is moderate-dose radiotherapy (40-50 Gy) and occasionally surgery. Adjuvant chemotherapy does not improve survival. Patients with EMP have a relatively low risk of progressing to multiple myeloma and have improved survival compared with those who present with SPB. CONCLUSION: Solitary plasmacytoma is an uncommon neoplasm that often arises in the head and neck. Optimal treatment is moderate-dose radiotherapy. Prognosis is relatively good and is better for patients with EMP compared with those presenting with SPB.