Merkel Cell Carcinoma, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology.

This selection from the NCCN Guidelines for Merkel Cell Carcinoma (MCC) focuses on areas impacted by recently emerging data, including sections describing MCC risk factors, diagnosis, workup, follow-up, and management of advanced disease with radiation and systemic therapy. Included in these sections are discussion of the new recommendations for use of Merkel cell polyomavirus as a biomarker and new recommendations for use of checkpoint immunotherapies to treat metastatic or unresectable disease. The next update of the complete version of the NCCN Guidelines for MCC will include more detailed information about elements of pathology and addresses additional aspects of management of MCC, including surgical management of the primary tumor and draining nodal basin, radiation therapy as primary treatment, and management of recurrence.

Basal Cell Skin Cancer, Version 1.2016, NCCN Clinical Practice Guidelines in Oncology.

Basal cell carcinoma (BCC) of the skin is the most common cancer, with a higher incidence than all other malignancies combined. Although it is rare to metastasize, patients with multiple or frequently recurring BCC can suffer substantial comorbidity and be difficult to manage. Assessment of risk is a key element of management needed to inform treatment selection. The overall management of BCC primarily consists of surgical approaches, with radiation therapy as an alternate or adjuvant option. Many superficial therapies for BCC have been explored and continue to be developed, including topicals, cryosurgery, and photodynamic therapy. Two hedgehog pathway inhibitors were recently approved by the FDA for systemic treatment of advanced and metastatic BCC, and others are in development. The NCCN Guidelines for Basal Cell Skin Cancer, published in full herein, include recommendations for selecting among the various surgical approaches based on patient-, lesion-, and disease-specific factors, as well as guidance on when to use radiation therapy, superficial therapies, and hedgehog pathway inhibitors.

Merkel cell carcinoma, version 1.2014.

Merkel cell carcinoma is a rare, aggressive cutaneous tumor that combines the local recurrence rates of infiltrative nonmelanoma skin cancer with the regional and distant metastatic rates of thick melanoma. The NCCN Guidelines for Merkel Cell Carcinoma provide recommendations on the diagnosis and management of this aggressive disease based on clinical evidence and expert consensus. This version includes revisions regarding the use of PET/CT imaging and the addition of a new section on the principles of pathology to provide guidance on the analysis, interpretation, and reporting of pathology results.

Dermatofibrosarcoma protuberans, version 1.2014.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor characterized by a relatively high risk of local recurrence and low risk of metastasis. The NCCN Guidelines for DFSP provide multidisciplinary recommendations on the management of patients with this rare disease. These NCCN Guidelines Insights highlight the addition of the Principles of Pathology section, which provides recommendations on the pathologic assessment of DFSP. Because DFSP can mimic other lesions, immunohistochemical studies are often required to establish diagnosis. Cytogenetic testing for the characteristic translocation t(17;22)(q22;q13) can also be valuable in the differential diagnosis of DFSP with other histologically similar tumors.

The historical evolution of the understanding of the submandibular and sublingual salivary glands.

The discovery of the major salivary glands was primarily a story of the establishment of the glands major excretory ducts. Occurring during the Renaissance: religious, political, and philosophical considerations played a role in defining the structure's function. We describe the history and background of these political, religious, and philosophical factors. Next, we present a translation of the original texts, describing the establishment of the submandibular and sublingual salivary glands. We place these translations into historical context and comment on their naming propriety. Initially we translate the works of the 15th century anatomists. Next, we look at the discovery and description of the submandibular gland's duct by Thomas Wharton (1614-1673) in his landmark book, Adenographia sive glandularum totius corporis descriptio (Adenographia or the description of the glands of the entire body) (1656). Next, the somewhat unexpected discovery of the duct of the parotid gland by Nicholas Stenson (1638-1686) and his publication, De glandulis oris et novis earundum vasis (On the glands of the mouth and their new ducts) (1661), done primarily for his discussion of the submandibular and sublingual gland anatomy. Finally we outline the description and discussion by Caspar Bartholin (1655-1738), in De ductu salivali, hactenus non descripto observatio anatomica (Anatomical observation of a salivary duct not hitherto described) (1685) of the origin of the complex drainage system of the sublingual gland. The honor of naming these glands rested with the discoverer of their ducts. All original works were published in Latin, and we have translated these texts to more fully understand the author's perspectives and historical context for a more interesting and complete story.

Historical vignettes of the thyroid gland.

Although "glands" in the neck corresponding to the thyroid were known for thousands of years, they were mainly considered pathological when encountered. Recognition of the thyroid gland as an anatomical and physiological entity required human dissection, which began in earnest in the 16th century. Leonardo Da Vinci is generally credited as the first to draw the thyroid gland as an anatomical organ. The drawings were subsequently "lost" to medicine for nearly 260 years. The drawings were probably of a nonhuman specimen. Da Vinci vowed to produce an anatomical atlas, but it was never completed. Michelangelo Buonarroti promised to complete drawings for the anatomical work of Realdus Columbus, De Re Anatomica, but these were also never completed. Andreas Vesalius established the thyroid gland as an anatomical organ with his description and drawings in the Fabrica. The thyroid was still depicted in a nonhuman form during this time. The copper etchings of Bartholomew Eustachius made in the 1560s were obviously of humans, but were not actually published until 1714 with a description by Johannes Maria Lancisius. These etchings also depicted some interesting anatomy, which we describe. The Adenographia by Thomas Wharton in 1656 named the thyroid gland for the first time and more fully described it. The book also attempted to assign a function to the gland. The thyroid gland's interesting history thus touches a number of famous men from diverse backgrounds.

The historical Latin and etymology of selected anatomical terms of the larynx.

The etymological evolution of the anatomical terms larynx, cricoid, glottis, epiglottis, and thyroid (cartilage) dates to antiquity. Human dissection replaced animal in the 16th and 17th centuries and terms evolved. This evolution was recorded in the literature largely in Latin. We translated key studies of laryngeal anatomy from the 16th century to better understand this evolution. We present the Latin with our translations, and historical commentary as essential to this understanding. Vesalius favored the Latin scutiform (shield) for the thyroid cartilage, but recognized peltalis (shield). The Basle Nomina Anatomica (BNA) chose the Greek thyroid (theta upsilon rho epsilon omicron epsilon iota delta eta) for modern convention. Vesalius used the name "innominate" for the cricoid cartilage, but described its resemblance to a ring, drawn in the margin of the Fabrica. Krikoid, the Greek for ring shaped, was adopted by the BNA. Although the term arytenoid was used for centuries, Vesalius argued the Greek name referred to the spout of a cup or ladle. He recognized the human arytenoids as two separate cartilages as opposed to single in certain animals. The glottis was defined by Vesalius as the vocal fold or rima glottidis of today, and he advanced its function by understanding the paired, mobile arytenoid cartilages. He defined the function of the epiglottis and first described the pre-epiglottic space. Vesalius' student at Padua, Italy, Columbo contributed to anatomical knowledge, but animosity between them clouded the record. Harvey, working 75 years later in England, offers an evolutionary window from Vesalius. Harvey's laryngeal studies preceded by a decade his groundbreaking studies on the circulation of blood.

Medical malpractice and sinonasal disease.

OBJECTIVE: Sinonasal disease is a common diagnosis that is encountered by nearly all specialties. This study examines medical malpractice trends in sinonasal disease. METHODS: One hundred fifty-two malpractice cases involving sinonasal disease between 1988 and 2005 were obtained from a computerized legal database. RESULTS: Defendants prevailed in 62 percent of the cases with a median monetary award of $650,000. Younger patients prevailed at a higher rate than did older patients (50% vs 35%), and men had a higher median award than did women ($1.0 million vs $314,000). These results approached but did not reach statistical significance (P = 0.09, P = 0.06). Otolaryngologists were the most commonly sued specialty (56%). The most common complications of endoscopic sinus surgery included cerebrospinal fluid leak, orbital trauma, and anosmia. Cancer plaintiffs received the highest median award of $1.5 million. CONCLUSIONS: Physicians must be diligent in forming differential diagnoses, and surgeons must ensure informed consent is obtained and documented. Future studies should continue to identify risk management strategies and areas for malpractice reform.

Medical malpractice and transoral robotic surgery: Evaluation and some preemptive thoughts.

Medical malpractice is costly and disruptive, and it is important to prevent. We conducted a study with the objective to look at medical malpractice in robotic surgery overall, to evaluate reasons for litigation, and to comment on possible strategies to avoid litigation with transoral robotic surgery. We used the Westlaw computerized database to identify all state and federal verdict summaries in medical malpractice cases. We found 17 cases alleging malpractice that involved the use of robotic surgery. In all, the plaintiffs in 6 cases (35%) contended that an open rather than a robotic approach should have been used, 5 (29%) alleged negligent credentialing, 4 (24%) alleged training deficiencies, 2 (12%) alleged manufacturing problems, and 1 (6%) charged that robotic surgery should have been performed instead of open surgery (1 case involved two of these allegations). In 11 cases (65%), plaintiffs charged that robotic surgery contributed to an undesirable outcome, and in 6 cases (35%) they raised concerns about informed consent. In all, only 5 of the 17 lawsuits (29%) resulted in plaintiff verdicts or settlements; damages ranged from $95,000 to $7.5 million. We believe the courts should not play a major role in establishing safety guidelines for the introduction of new technology such as robotic surgery. Instead, training and credentialing guidelines should be established by appropriate national associations and societies to assist hospitals in doing so.

Medical malpractice and cancer of the skin.

BACKGROUND: A litigation crisis exists in this country, and it may be better understood through analysis of lawsuits. Verdict summary analysis has been used for this purpose. METHODS: Ninety-nine jury verdict reviews from 1986 to 2001 were obtained from a computerized database. Reviews compiled data on defendants, plaintiffs, allegations of wrongdoing, cancer cell types, sites, outcomes, and case summaries. Data was entered into a spreadsheet for analysis. RESULTS: Failure to diagnose was alleged in 54%; of these, 48% alleged biopsies were inappropriately omitted. Allegations were highest for failure to diagnose in dermatologists and general practice physicians, for misdiagnosis in pathologists, and for complications in surgeons. Young patients and those with poor outcomes received more and larger awards. Trauma appears to have support for a role in causation of skin cancer in some courts. CONCLUSIONS: The study of skin cancer suits may help develop risk management and prevention strategies.

Medical malpractice and cancer of the larynx.

OBJECTIVE: To analyze the frequency, clinical characteristics, and legal outcomes of malpractice litigation initiated by patients with cancer of the larynx. STUDY DESIGN: Retrospective review of 23 jury verdict reports from 11 states. METHOD: Jury verdict reviews from 1976 to 1997 were obtained from a computerized legal database compiled from all state and federal civil court decisions. Reviews compile pertinent data on defendants, plaintiffs, verdict outcomes, indemnity payments, allegations of wrongdoing, and provide case summaries. RESULTS: Delays in diagnosis were alleged in 19 of 23 (83%) suits. Hoarseness was present in 10 of 19 (53%), a neck mass in 3 of 19 (16%), and no biopsy was taken in 11 of 19 (58%) of those delayed. Mean age was 47 years, with a peak incidence in the general population of 70 to 74 years. Laryngectomy was alleged to be a result or complication of delay in 12 of 19 (63%). Over half of the patients delayed received compensation, and over half of the defendants were in general practice. Outcome was poor with a 35% mortality and a total of 47% either dead of disease or alive with disease. Complications, incorrect diagnosis, and informed consent did not play a significant role in initiating litigation. CONCLUSIONS: The delayed diagnosis of cancer of the larynx associated with litigation is frequently seen in younger patients presenting with hoarseness or neck masses. These patients frequently have poor outcomes. Patients with symptoms of cancer of the larynx must be aggressively evaluated regardless of age. Risk management goals to prevent delays in diagnosis may help prevent subsequent litigation.

Cancer of the oral cavity and medical malpractice.

OBJECTIVE: To analyze malpractice litigation initiated by patients with cancer of the oral cavity as a result of allegations concerning their diagnosis and treatment. STUDY DESIGN: Retrospective review. METHODS: Jury verdict reviews from 1984 to 2000 were obtained from a computerized legal database and analyzed. Reviews compile pertinent data on defendants, plaintiffs, verdict outcomes, indemnity payments, and allegations of wrongdoing and provide case summaries. Fifty cases from 21 of all 50 states were obtained. Data were entered into a spreadsheet for analysis. RESULTS: Overall, a young age (mean age, 45 y) and poor oncological outcome (47% dead) were seen. The younger age group (<47 y) had a better outcome (65% without disease), were more often misdiagnosed (19%), won awards (60%), and had higher awards (average award, $755,824) as compared with those older than 47 years of age, who had 39% without disease, 0% misdiagnosed, and 52% who won awards that were less, on average, at $495,417. Failure to diagnose the cancer was alleged, overall, in 43 of 50 cases (86%), and in general practice, dentists, and otolaryngologist in 100%, 85%, and 89%, respectively. Suits alleging a delay in diagnosis of less than 3 months were defendant verdicts in 86%, and of more than 3 months, in 40%. Failures to perform biopsy and to refer were common allegations. CONCLUSIONS: Young age of patients bringing litigation is seen in patients with oral cancer. These patients frequently have poor oncological outcomes. Risk management goals to prevent delays in diagnosis may help prevent subsequent litigation.

Medical malpractice and facial nerve paralysis.

BACKGROUND: Iatrogenic facial nerve paralysis is a devastating surgical complication that occasionally results in litigation. OBJECTIVE: To analyze litigation trends to better understand the causes and outcomes of suits involving facial nerve paralysis to prevent future litigation and improve physician education. DESIGN: Retrospective review. SETTING: All US civil trials. PARTICIPANTS: All state and federal civil trials alleging malpractice and facial nerve paralysis were reviewed. METHODS: Jury verdict reviews from January 1, 1985, to December 31, 2000, were obtained from a computerized legal database and analyzed. Reviews compile data on defendants, plaintiffs, allegations of wrong doing, and expert witness specialties, and provide case summaries. Fifty-three cases from 19 of the 50 states were obtained. Data were entered into a spreadsheet for analysis. MAIN OUTCOME MEASURES: Verdicts and indemnity payments. RESULTS: Suits reviewed were as follows: cosmetic, 12 (23%); otologic, 13 (25%); nonneoplastic disease of the parotid or other benign conditions of the head and neck, 15 (28%); benign neoplasms of the parotid, 9 (17%); malignant neoplasms of the parotid, 1 (2%); and temporomandibular joint operations, 3 (6%) (percentages do not total 100 because of rounding). Allegations of negligence were frequently multiple and included lack of informed consent (16 [30%] of 53), failure to diagnose (10 [19%] of 53), and surgical misadventures (47 [89%] of 53). Excluding failure to obtain consent or to diagnose, 28 suits still alleged negligence based solely on a surgical misadventure. Of these suits, 12 (63%) resulted in plaintiff awards. CONCLUSIONS: Surgeons must emphasize and document the likelihood and consequences of this devastating complication to all patients undergoing surgery in this area. Risk management goals include a thorough and timely examination and careful and thoughtful surgical approaches. However, patient rapport and bedside manner may be the only protection the surgeon has from litigation arising from this complication.

Medical malpractice and head and neck cancer.

PURPOSE OF REVIEW: The purpose of this review is to evaluate medical malpractice involving patients with head and neck cancer. In the United States, problems associated with the medical malpractice environment are reaching proportions that threaten the delivery of health care. RECENT FINDINGS: Several methods to learn more about the trends and ramifications of litigation have been developed. Although patients with cancer of the head and neck bring suits rarely, when they do several themes seem prevalent. Delays in diagnosis are common allegations. Plaintiffs are frequently much younger than expected, and the oncologic outcome is frequently poor. Defendant physicians occasionally unwittingly add to the delay by not expecting the younger patient. The poor oncologic outcome may be related to the delay or to a biologically more aggressive disease. Relationships between these factors are explored. Consent issues are also relatively common. SUMMARY: Physicians must strive to know as much as possible about tort reform, and to contribute to the process. Litigation analysis and other tools to expand our understanding can be used to educate ourselves and the legal community. Scientific standards must be developed along with clinical pathways to guide ourselves to prevent litigation. They may also guide the legal community to establish more rational standards of care based on a consensus of expert opinions.

The influence of the final cause doctrine on anatomists of the sixteenth and seventeenth centuries concerning selected anatomical structures of the head and neck.

The Doctrine of Final Cause, taken from Aristotle's "causes" and modified by Claudius (Aelius) Galen (of Pergamon) stated that for an anatomical part to exist it must have a "cause," not an end point, but a purpose or goal, natural or divine. This affected the renaissance anatomist's thinking. We explore this doctrine's relationship with human head and neck anatomy from antiquity's Aristotle and Galen, and the leading renaissance anatomists from the 16th and 17th centuries. Their relevant writings were influenced by religious and political beliefs and varied from humanistic to reactionary. Tracing anatomical controversies through these works reveal the humanism of Vesalius and others as paralleling the humanists of art and literature. These controversies illustrate how the body was used to demonstrate function, uses, and causes from higher sources. Humanists advanced the social, philosophical, intellectual, literary, and medical/anatomical thoughts of this period. They stood between the Christian church of the Middle Ages and modern science. Like religion, medicine and anatomy had its own revealed sources of knowledge and had sacred texts like Galen's. Vesalius' the Fabrica and the woodcuts established suddenly the beginning of modern observational science and art as the direct and faithful representation of natural phenomena. They displayed anatomy such that others could understand, including errors of Galen, bringing Vesalius into ecclesiastical conflict. Evolutionary scientists today see mutations as favorable or unfavorable depending on the environment. Mutations are random or directed by divine plan, according to perspectives of this ancient debate.

Cetuximab in hemodialysis: a case report.

BACKGROUND: Concurrent chemoradiotherapy with cisplatin is the standard therapy for patients with unresectable locally advanced head and neck squamous cell carcinoma. However, cisplatin administration in patients on hemodialysis is complicated by the need to perform hemodialysis immediately after the infusion. Concurrent chemoradiation with cetuximab has been approved in definitive treatment of locally advanced head and neck cancer. Although cetuximab is not excreted via the kidneys, its use in patients on hemodialysis has not been reported. METHODS AND RESULTS: We present the case of a 65-year-old man undergoing hemodialysis, with stage IVA squamous cell carcinoma of the hypopharynx. Given the logistics of performing hemodialysis immediately postcisplatin, he received concurrent chemoradiotherapy with cetuximab. He tolerated treatment well with minor side effects. CONCLUSION: Cetuximab can be safely used in patients with renal impairment. This is the first reported case of the use of cetuximab in a patient undergoing hemodialysis.

Litigation and the lingual nerve.

PURPOSE: A "malpractice crisis" exists in the United States. Litigation analysis helps to understand the causes and may be useful in prevention of suits. This study reviews litigation regarding the lingual nerve. MATERIALS AND METHODS: Jury verdict reports were obtained from a computerized legal database for the years 1987 through 2000. The study reviews all state and federal civil trials in the United States. Reviews compile information on plaintiffs and defendants, allegations of wrong-doing, reasons for litigation, anatomic sites of injuries, specialties of expert witnesses, verdict results, and awards received. RESULTS: Thirty-three suits from 12 states were obtained. Dentists or oral surgeons were involved in 87%, and otolaryngologists were involved in 13% of suits. Tooth extractions were involved in 79%, and 50% of these resulted in financial awards. Lack of informed consent was alleged in 52% of suits overall and in 46% of tooth extraction suits. Expert witnesses were of the same specialty for both sides in 81%. Inadequate training and selection of the wrong surgical approach were alleged in 18% and 15%, respectively. Anatomic variations were thought to be present in 15%. CONCLUSIONS: Surgeons must be aware of anatomic variations and regions in which injury to the lingual nerve frequently occurs. Written informed consent may help decrease litigation in known risk regions.

Medical malpractice and the thyroid gland.

BACKGROUND: A medical malpractice litigation "crisis" exists in this country. Analyzing litigation trends through verdict summaries may help understand causes. METHODS: Jury verdict reviews from 1987-2000 were obtained from a computerized database. Reviews compile data on defendants, plaintiffs, allegations of wrongdoing, and verdict summaries. RESULTS: Thirty suits from nine states occurred. Plaintiffs were women in 80% of the cases, with a median age of 41. Fifty percent of patients (15 of 30) had a bad outcome, (9 of 30 dead, 4 of 30 with neurologic deficits, 1 blind, and 1 alive with cancer). Thirty percent alleged surgical complications, mostly recurrent laryngeal nerve injury, and 75% of cancer patients alleged a delay, either through falsely negative biopsies or no biopsy taken. Respiratory events occurred in 43% and frequently resulted in large awards. CONCLUSIONS: The liberal use of fine-needle aspiration and documentation of surgical risks may help reduce litigation. Complications and bad outcomes do not indicate negligence. Analysis may contribute to risk management strategies or litigation reform.