Purpose Merkel cell carcinoma (MCC) is a rare cutaneous malignancy. Because of the absence of randomized studies, the real benefit of adjuvant radiation therapy in MCC is unclear. The aim of this study was to better define the role of adjuvant radiation therapy in the management of MCC. Methods The Surveillance, Epidemiology, and End Results (SEER) survey from the National Cancer Institute was queried from 1973 through 2002. Retrospective analysis was performed. The end point of the study was overall survival. Results There were 1,665 cases of MCC in the SEER registry. Presentation by stage were 55% stage I, 31% stage II, and 6% stage III. Eight percent of the cases could not be staged because of incomplete data. Surgical intervention was a component of therapy in 89% of the cases (n = 1,487). The median survival for the entire cohort was 49 months, and median follow-up was 40 months. Adjuvant radiation was a component of therapy in 40% of the surgical cases. The median survival for those patients receiving adjuvant radiation therapy was 63 months compared with 45 months for those treated without adjuvant radiation. The use of radiation was associated with an improved survival for patients with all sizes of tumors, but the improvement with radiation use was particularly prominent when analyzing those patients with primary lesions larger than 2 cm. Conclusion The use of adjuvant radiation therapy is associated with improved survival in patients with MCC. Prospective evaluation of adjuvant radiation therapy in this setting is warranted.

Adjuvant radiation therapy is associated with improved survival in merkel cell carcinoma of the skin

A classically identified “notochordal” cell population in the nucleus pulposus is thought to regulate disk homeostasis. However, the embryonic origin of these cells has been under dispute for >60 years. Here we provide the first direct evidence that all cell types in the adult mouse nucleus pulposus are derived from the embryonic notochord. Additionally, rare isolated embryonic notochord cells remained in the vertebral column and resembled “notochordal remnants,” which in humans have been proposed to give rise to a rare type of late-onset cancer called chordoma. Previously, this cell type had not been identified in the mouse model system. The development and characterization of a mouse model that can be used to fate map nucleus pulposus precursor cells in any mutant background will be useful for uncovering the cellular and molecular mechanisms of disk degeneration. In addition, the identification of notochordal remnants in mice is the first step towards generating an in vivo model of chordoma. Developmental Dynamics 237:3953–3958, 2008. © 2008 Wiley-Liss, Inc.

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Identification of nucleus pulposus precursor cells and notochordal remnants in the mouse: Implications for disk degeneration and chordoma formation

Among various types of ion species, carbon ions are considered to have the most balanced, optimal properties in terms of possessing physically and biologically effective dose localization in the body. This is due to the fact that when compared with photon beams, carbon ion beams offer improved dose distribution, leading to the concentration of the sufficient dose within a target volume while minimizing the dose in the surrounding normal tissues. In addition, carbon ions, being heavier than protons, provide a higher biological effectiveness, which increases with depth, reaching the maximum at the end of the beam's range. This is practically an ideal property from the standpoint of cancer radiotherapy. Clinical studies have been carried out in the world to confirm the efficacy of carbon ions against a variety of tumors as well as to develop effective techniques for delivering an efficient dose to the tumor. Through clinical experiences of carbon ion radiotherapy at the National Institute of Radiological Sciences and Gesellschaft fur Schwerionenforschung, a significant reduction in the overall treatment time with acceptable toxicities has been obtained in almost all types of tumors. This means that carbon ion radiotherapy has meanwhile achieved for itself a solid place in general practice. This review describes clinical results of carbon ion radiotherapy together with physical, biological and technological aspects of carbon ions.

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A review of update clinical results of carbon ion radiotherapy.

Between 1962 and 1978, 15 patients presenting with a solitary plasmacytoma were treated with curative intent by radiotherapy alone at the University of Florida. Criteria for admission to this study were: 1) a biopsy-proven, apparently solitary focus of plasmacytoma; 2) bone marrow biopsy showing less than 10% plasma cells; and 3) no evidence of disseminated disease. In 9 patients the primary site was osseous and in 6 patients extramedullary; 5 of the 6 extramedullary lesions were located in the upper respiratory passages. Two of the 6 extramedullary plasmacytomas progressed to multiple myeloma at 2 and 7 months. The remaining 4 patients have been disease free for periods ranging from 2 2 3 years to over 16 years. Of the 9 patients with osseous lesions, 3 developed multiple myeloma in 3–23 months and one developed a solitary second bone lesion at 9 years. One patient with a large sacral lesion developed a local recurrence following an initial radiation dose of 3000 rad. The recurrence was re-treated with radiation, and local control was obtained. No other local failures occurred. This study presents a detailed analysis of the time-dose relationship required for local control, based on a study of our own patients and a review of the literature.

Solitary plasmacytoma of bone and soft tissue

A small proportion of patients with plasma cell myeloma have a solitary plasmacytoma of bone. Strict staging criteria, including normal MR imaging studies of the axial skeleton and the long bones and absence of monoclonal plasma cells detected by flow cytometry or PCR, are required for diagnosis. Radiotherapy at a dose of 4500 cGy is required to eradicate the local tumor. Many patients enjoy prolonged disease-free survival, but the incidence of systemic relapse is high. It is expected, however, that if strict diagnostic criteria are applied some patients may be cured. Extramedullary plasmacytoma is an even rarer plasma cell disease which usually occurs in the head and neck area. Careful microscopic and immunohistochemical studies are required for the correct diagnosis, because this disease can be confused with other malignancies, particularly lymphomas. The treatment of choice is radiotherapy which, in cases of head and neck plasmacytomas, should encompass the adjacent lymph nodes. Most patients with extramedullary plasmacytoma can be cured, and fewer than 30% develop a distant failure in the form of multiple myeloma or multiple extramedullary tumors.

https://www.hemonc.theclinics.com/article/S0889-8588(05)70124-6/pdf

Solitary plasmacytoma of bone and extramedullary plasmacytoma

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.

Salivary Gland Pleomorphic Adenoma

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. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX–XXX, 2005

Adult head and neck 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%.

The Management of Adult Soft Tissue Sarcomas

Solitary plasmacytoma of bone and soft tissues.

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 com- plications, 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 approx- imately 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. A 2005 Wiley Periodicals, Inc. Head Neck 27: 159-165, 2005

Charles M. Mendenhall

Papers

Salivary Gland Pleomorphic Adenoma

Adult head and neck soft tissue sarcomas

The Management of Adult Soft Tissue Sarcomas

Solitary plasmacytoma of bone and soft tissues.

Skull base chordoma.