Law enforcement is a unique occupation in many ways. The majority of law enforcement officers wear a uniform while working; many have grooming standards and conduct regulations to which they must adhere; some have sworn to uphold laws with which they do not necessarily agree. For example, the federal Free Access to Clinic Entrance, or FACE, law criminalizes attempts to interfere with a woman’s access to an abortion clinic. Enforcing the laws also is, by its very nature, a job requiring continuous staffing–24 hours a day, 7 days a week, 365 days a year. These unique aspects of the profession sometimes cause personal conflicts with individual employees’ religious beliefs. For example, what happens when a uniformed patrol officer feels it is a religious duty to violate the department’s ban on lapel pins by wearing a Christian cross lapel pin or when a group of Muslim male officers violate a department’s “no facial hair” policy by growing beards as required by their religion? What about the captain who refuses to assign officers to maintain order at the sight of an abortion clinic protest because his Catholic faith frowns upon abortion? Finally, how should a department handle a potential scheduling nightmare when its officers raise objections to shift assignments conflicting with their Sabbaths or days of worship? This article addresses these issues and raises awareness of the myriad legal provisions that should govern handling them.

Massachusetts and Delaware are two states that have climbed aboard the collaboration wagon, taking steps to make electronic health records (EHRs) a universal reality. In Massachusetts, 34 organizations known as the Massachusetts eHealth Collaborative, which includes many of the state’s major delivery systems and insurers, have pledged to build a statewide health information network. The entity will promote widespread implementation of clinical information systems, including EHRs, clinical decision support and data exchange applications. The eHealth Collaborative emanated from a 2004 Blue Cross Blue Shield of Massachusetts pledge of up to $50 million for an effort to bring uniform EHR technology to every hospital and healthcare system in the state.
On a smaller scale, federal funding to the tune of $700,000 is earmarked for the Delaware Health Information Network (DHIN), in which key hospitals, doctors, laboratories and other medical providers throughout Delaware will help the State transition to an EHR system.

Throughout every facility that studies and treats patients with cancer, there are copies-in departments of pathology, surgical and medical oncology, diagnostic and radiation oncology, surgical oncology, and cancer registries-of the 5th edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual. There should also be a copy of the International Union Against Cancer (UICC) TNM Supplement: A Commentary on Uniform Use, 2nd edition, edited by Wittekind, Henson, Hutter, and Sobin, UICC, 2001. The AJCC manual does not differ from the TNM Classification of Malignant Tumours manual; they were formulated together but are published as separate books. Both manuals are in their 5th editions and were most recently published in 1997.
As well written as the 5th edition of the AJCC Cancer Staging Manual is, this commentary is virtually essential for its optimal use, as was the 1st edition of this supplement, which was published in 1993. The supplement is a short 140-page book that is divided into the following sections: explanatory notes, both general and specific (anatomic); site-specific recommendations for pT and pN; new tumor– node-metastasis (TNM) classifications recommended for testing; and optional proposals for testing new “telescopic” ramifications of TNM. An additional section featuring 14 pages dedicated to answering frequently asked questions on the TNM Web site is possibly the most valuable section of the book. This supplement is a warehouse of information. Among the topics discussed are how to code gastrointestinal sarcomas and malignant lymphomas, as well as malignant thymomas. In many institutions, there are arguments regarding subdivisions of existing standard TNM categories, some based on anecdote, others on solid analyzed data. The chapter on telescopic ramifications is very instructive, full of suggestions on how to collect additional data without altering the definitions of existing TNM code sets.
If you read only one chapter in the supplement, it should be the “Frequently Asked Questions” chapter. Even experienced coders will profit from reading this text. Questions answered include how does one use the R classification? If there is residual tumor after surgery, is it always stage IV? If I am not sure of the correct TNM category (because of unclear measurements), which TNM do I select? If tumor is spilled into the abdomen or pleural cavity during surgery, how does this affect classification? The 4th edition of the TNM manual had a classification for brain tumors; why doesn’t the 5th edition? Who is ultimately responsible for TNM coding?

Simply stated, if we do not code, collect, collate, and study TNM coding data sets, we will not know whether our diagnostic and therapeutic modalities are any improvement on the old. That is why this book is so important.

I have 2 final comments. There is a related classification of neoplasms, the World Health Organization International Classification of Disease for Oncology (ICD-O), 2nd edition, first published in 1958. The morphology nomenclature is identical to that of the M field for neoplasms in the Systematized Nomenclature of Medicine (SNOMED), published by the College of American of Pathologists. To facilitate appropriate comparison of data, I recommend that ICD-O classification also be performed.

Lastly, this reviewer would like to observe that even with widespread uniform TNM and ICD coding, we shall still be a considerable way from where we need to be to truly analyze the information in the charts of our patients with cancer. When, and until, there is a method to code the entire medical record for its clinical content-linked to or “cross-walked” to all of the other classifications, such as TNM, ICD, nursing, pharmacologic, and other classifications-we will not be in a position to truly “mine” the record for its most precious gems. The Veterans Administration System, with its electronic medical record, and the Kaiser-Permanente system, with its SNOMED-CT (SNOMED– Clinical Terms) project, are leaders in this area. Let us hope that in the near future we will have a way for all of us to move toward the goal of a clinically coded, electronic medical record.

As mentioned previously in Washington Report, the push is on to reduce medical errors in the US. The focus has now shifted to Congress. A bipartisan coalition of senators recently urged lawmakers to approve a bill to require healthcare facilities to establish safety procedures and report instances of serious error. The bill’s sponsors, Senators Charles Grassley, R-IA; Joseph Lieberman, D-CT; Bob Kerrey, D-NE; and Richard Bryan, D-NV, want to reduce the medical error rate by 50% over the next 5 years.
The measure known as SAFE (Stop All Frequent Errors in Medicare and Medicaid Act of 2000) is based on recommendations from the Institute of Medicine report published last November. Among other things, the institute found that as many as 98,000 patients die every year because of medical mistakes.

SAFE also would establish a Center for Patient Safety within the Agency for Health Research and Quality. Designed to improve safety and reduce the incidence of medical errors, the Center would conduct research and dispense grants related to medical error reduction. It also would establish national goals for patient safety and mechanisms to track such goals.

The measure would target healthcare purchased through Medicare and Medicaid. Facilities that want to participate in these 2 federal programs, including hospitals, critical access hospitals, skilled nursing facilities, and home health agencies, would have to meet the requirements of the act.
“Though Medicare and Medicaid, we can work to reduce medical errors in almost every health facility in the country,” said Sen. Lieberman.

SAFE would require health institutions to establish a medical safety program that produces measurable reductions in error. The program would require the reporting of deaths and serious injuries and the identification of their causes. Under the proposal, health institutions would have to take steps to prevent further accidents.

When asked about the role of laboratories in all this, American Clinical Laboratory Association president David Sundwall, MD, said, “ACLA is very interested in it. We think it presents an opportunity for labs to get some good press because we have a very good record and a good story to tell.” He added that research indicates that medical errors occur because of system failure. “Laboratories believe they can help with those system problems.”

Under the bill, medical institutions would have to report incidence of errors to state public health departments, Medicare peer review organizations, and relevant accrediting organizations. Facilities would then select one of those three entities to oversee whether they are complying with the law’s requirements. The information concerning errors would be stripped of identifiers, then provided in aggregate form to federal officials for research and other purposes.

The Department of Health and Human Services would not publicize the name of an institution unless the facility demonstrates a pattern of poor performance for more than 2 years, and fails to comply with the reporting requirements and to take steps to address safety problems.

While there is growing public support for efforts to reduce medical mistakes, groups representing physicians and hospitals are concerned that the mandatory reporting of errors would result in additional lawsuits. To win passage, Capitol Hill experts say, lawmakers will have to come up with a way that provides physicians and hospitals with protections from legal action.

Regional carriers still on drawing board

HCFA is indicating that it plans to phase in a regional carrier system to process Medicare Part B laboratory claims as required by the 1997 Balanced Budget Act. But the phase-in is not expected to start until sometime next year. The use of regional carriers as well as uniform lab payment policies are intended to make the handling of lab claims more consistent across carrier areas.

Under the budget act, up to 5 regional carriers were scheduled to be in place by July 1, 1999. But HCFA has taken no action to get the effort going. “We think this is long overdue and we are not happy with the delay,” says ACLA’s Sundwall.

HCFA maintains that the delay was due in part to year 2000 compliance work. But Sundwall says that some HCFA staffers have reservations about the regional carrier requirement. Even so, the HHS Office of Inspector General has said it favors the use of regional carriers to handle lab claims. The OIG believes that the regional carrier approach has worked well for durable medical equipment claims.

Providing handwashing, rinsing, and sanitizing, fully automated Radius(TM) performs 10-sec cycle using non-alcohol disinfecting solution and high-pressure water jets that ensure uniform hand wash. Technology virtually eliminates transient pathogens from hands and increases frequency of employee handwashing. Combining RFID employee badge reading with data-reporting software, touchless system provides hospitals with automated monitoring and reporting of handwashing events.

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The new Radius(TM) system is the first fully-automated handwashing method available for preventing healthcare-acquired infections.
GOLDEN, Colo., June 18 / — Resurgent Health and Medical will introduce the Radius(TM) Automated Handwashing and Monitoring System at the Association for Professionals in Infection Control and Epidemiology (APIC) 2007 Annual Conference, June 24 - 28 in San Jose, Calif.

The new Radius system replaces the inconvenience and inconsistency of manual handwashing with a totally automated approach to infection prevention. It is the first fully-automated, touchless system available in healthcare for mechanical handwashing, rinsing and sanitizing. It performs a ten-second cycle using a non-alcohol disinfecting solution.

By making handwashing quick and easy to perform, the Radius system increases healthcare workers’ compliance to a hand hygiene program. The system further boosts compliance by ensuring a pleasant, uniform handwash using high-pressure water jets that perform a consistent wash-and-sanitize cycle every time the machine is used.
Handwashing compliance can be measured automatically using the Radius system. Its patent-pending radio-frequency identification (RFID) employee badge reading technology, combined with its data-reporting software, provide hospitals with automated monitoring and reporting of handwashing events.

The Radius system’s proprietary rotating handwash cylinder technology has been proven through 20 years of successful experience in food processing, food handling and cleanroom manufacturing. According to industry studies, the technology virtually eliminates transient pathogens from the hands and increases the frequency of employee handwashing by up to 300%.

The technology is also shown to use up to 75% less water than manual handwashing, discharge 75% less wastewater, and reduce waste in soap utilization.

“We’re committed to helping achieve ‘zero tolerance’ in the prevention of healthcare-acquired infections,” says Jim Glenn, CEO of Resurgent Health and Medical. “Our new Radius system brings to healthcare a proven technology that has enabled other critical industries to achieve near-perfect elimination of infectious workplace pathogens. Furthermore, the Radius system introduces a total prevention approach by automating measurement and documentation of handwashing compliance.”

IN AUGUST 2004 a Houston, Texas, man with fatal liver cancer received a transplant as a consequence of a national advertising campaign that documented his plight and requested families to specifically designate him as the recipient of any available liver. Although organ donors or their families can specify recipients (which is unusual and generally occurs only within families), the individual’s initiative violated the spirit of the Uniform Anatomical Gift Act, which proscribes the buying and selling of organs. No money was exchanged, however, so the transaction was legal. But the intent of the law is to make medical considerations the sole criteria for organ distribution, and the considerable resource expenditure involved in procuring the precious liver in this case was an end run that legislators hadn’t foreseen.
In the commentary section of the September 1, 2004, Chicago Tribune, three bioethicists describe the mostly egalitarian nature of the U.S. system for distributing livers for transplant. Noting the loophole in the law that allowed the Houston man to essentially purchase his “new” liver, they call for tightening the law governing organ donation.

At first glance it’s hard to argue with this policy of fairness. But this viewpoint flies in the face of the direction in which U.S. healthcare has been evolving for the last ten or fifteen years. Medical care has become a big business and it increasingly functions in a way that is based on the principles of the marketplace. This means that people can access healthcare services in proportion to their ability to pay for them. And, since the cost of healthcare is skyrocketing, an increasing number of Americans can’t afford it.
This dreary fact is played out in the medical marketplace daily. In an effort to safeguard their precarious bottom lines, healthcare providers avoid patients who can’t pay or who have inadequate insurance. Hospitals, for instance, though obligated by law to treat all patients in emergency situations, are quick to transfer indigent or uninsured patients to public hospitals as soon as the patients are stabilized. Furthermore, many physicians refuse to see Medicaid insured patients because the poor reimbursement they receive doesn’t cover expenses. Even the number of physicians who accept Medicare is decreasing.

The meteoric rise in the cost of medical insurance is a subtler competitive force that makes healthcare hard to obtain for low wage earners. Premiums have increased almost 25 percent in the last two years alone. To remain competitive in their respective industries, employers increase employee contributions to health benefits and purchase plans with ever larger deductibles and co-pays. Especially among small businesses, the number of employers who offer any kind of health insurance is declining. Only 63 percent of businesses with two hundred or fewer employees currently offer health benefits, down from 68 percent in 2001. Even when health insurance is offered as a benefit, an increasing number of workers find the employee contribution prohibitive.

The price of prescription drugs also cuts people out of the medical marketplace. One reason for the extraordinary expense of drugs is the aggressive marketing practices of the pharmaceutical industry. It spends more than 10 percent of its budget on advertising alone, accounting for at least $17 billion of healthcare costs in the United States and inflating the cost of crucial medications to the point that many patients can’t afford them.

There are many more examples of common marketplace practices that help explain why people are unable to get adequate healthcare. In its embrace of marketplace principles, the healthcare industry is working like any other in a marketplace economy: its goods and services go to those who can afford them.

In this light, the Houston man’s successful campaign to get a liver conforms to the rules of engagement that have become the norm in the peculiarly American game of procuring medical services. Americans accept the notion of winners and losers in the acquisition of most goods and services. Accordingly, as healthcare costs continue their staggering rise, fewer Americans will be able to access even mainstream medical help, much less something as exotic as a liver transplant. If a liver or any other scarce resource goes to the highest bidder, then healthcare is working just like any other American capitalistic venture.

The bioethicists who feel that the Houston man “cut in the ling” have an alternative concept of how to deal with precious healthcare resources. They end their commentary by saying that “faced with scarcity … the right thing to do is what is right for all, not right for one.” Most developed nations endorse this philosophy and back it up with a social welfare system that provides basic care for all. The United States stands nearly alone in treating healthcare like any other commodity.

Data Synthesis.-We review the features of new, useful or potentially applicable marker antibodies as well as the new uses of already established antibodies in the area of diagnostic neuro-oncologic pathology, focusing on the use of IHC for differential diagnosis and prognosis.We discuss (1) placental alkaline phosphatase, c-Kit, and OCT4 for germinoma, (2) µ-inhibin and D2-40 for capillary hemangioblastoma, (3) phosphohistone-H3 (PHH3), MIB-1/Ki-67, and claudin-1 for meningioma, (4) PHH3, MIB-1/Ki-67, and p53 for astrocytoma, (5) synaptophysin, microtubuleassociated protein 2, neurofilament protein, and neuronal nuclei for medulloblastoma, (6) INI1 for atypical teratoid/ rhabdoid tumor, and (7) epithelial membrane antigen for ependymoma. All the markers presented here are used mainly for supporting or confirming the diagnosis, with the exception of the proliferation markers (MIB-1/Ki-67 and PHH3), which are primarily used to support grading and are reportedly associated with prognosis in certain categories of brain tumors.
Conclusions.-Although conventional hematoxylin-eosin staining is the mainstay for pathologic diagnosis, IHC has played a major role in differential diagnosis and in improving diagnostic accuracy not only in general surgical pathology but also in neuro-oncologic pathology. The judicious use of a panel of selected immunostains is unquestionably helpful in diagnostically challenging cases. In addition, IHC is also of great help in predicting the prognosis for certain brain tumors.

(Arch Pathol Lab Med. 2007;131:234-241)

Although conventional hematoxylin-eosin (H&E) staining is crucial for diagnosis, diagnostic neuropathology has benefited in the last 2 decades from the incorporation of, and recent advances in, immunohistochemistry (IHC). A number of markers for IHC have been developed in the area of diagnostic neuro-oncology, since glial fibrillary acidic protein (GFAP), the antibody against which is currently most commonly used in practice, was found by Eng et al1 in 1971 and was later reported as a useful marker antigen for astroglial cells by Kleihues et al2 in 1987.

In general, brain tumors are classified into 2 major groups, primary and metastatic, and the primary brain tumors can be classified further into 3 groups: neuroepithelial (eg, astrocytic, oligodendroglial, ependymal, choroid plexus, neuronal, and pineal parenchymal tumors), nonneuroepithelial (eg, meningioma, nerve sheath tumors, malignant lymphoma, pituitary adenoma, and germ cell tumors), and others (ie, tumors of unknown origin, eg, capillary hemangioblastoma). In neuropathology practice, we routinely use several useful IHC markers that are relatively sensitive and specific for some of these tumors (eg, GFAP for astrocytomas, synaptophysin for neuronal tumors); however, none of these are diagnostic (ie, no absolute sensitivity and specificity).

There have been many recent publications in the area of IHC in brain tumor pathology, with several articles relating to new specific antibodies.3-10 This review will discuss the features of new, reportedly sensitive and specific marker antibodies as well as new uses of already established antibodies in the areas of adult and pediatric, diagnostic neuro-oncology practice, based on recently published reports and our own experience.

GERMINOMA: PLACENTAL ALKALINE PHOSPHATASE, C-KIT (CD117), AND OCT4

Germinoma occurs predominantly in the pineal and suprasellar regions and is composed of lobules or sheets of uniform cells with large vesicular nuclei, prominent nucleoli, well-defined cell boundaries, and abundant clear cytoplasm, admixed with lymphoplasmacytic infiltrates. Given that, characteristically, intracranial germinomas are highly radiosensitive and chemosensitive, allowing for a high cure rate with radiation alone or cisplatin-based chemotherapy followed by low-dose radiotherapy, an accurate diagnosis is critical for patient management. The histologic features are virtually diagnostic when the specimens are sufficient for evaluation and are well preserved without artifact. Immunohistochemistry is of particular use in cases when either the specimen is very small or the lymphocytic infiltrate is predominant.11 As with ovarian dysgerminomas and testicular seminomas, intracranial germinomas are known to show immunoreactivity for placental alkaline phosphatase (PLAP) in a surface membrane or, somewhat less commonly, diffuse cytoplasmic distribution.12 This antigen is a cell surface glycoprotein and is normally expressed in syncytiotrophoblasts and primordial germ cells.13 Although this marker is the mainstay in current neuropathology practice, it has its shortcoming in that PLAP labeling is not a constant feature with variable sensitivity, intensity, and extent of reactivity, 3,12,14 and it can sometimes be difficult to interpret, especially in the cases with heavy inflammatory cell infiltrates and in specimens that were previously frozen.

These men ore the Soldiers of the 13th Sustainment Command (Expeditionary) mostly from Fort Hood, Texas, commanded by Brigadier General Michael J. Terry, Their mission is to provide combat service support to more than 200,000 soldiers throughout Iraq. They are a small part of the 20,000 soldiers providing logistical support to sustain the troops.

About four years ago, James Amps took up the sport of golf as a means to de-stress and to increase networking opportunities with potential participants for his project, A.M.P.S. Entrepreneurship/Leadership Institute. The Institute organizes retreats and workshops where teenagers, adults, teachers, youth leaders and parents are partnered with community and business leaders who, over the course of the event, are equipped with financial and leadership development tools which they can carry with them throughout their lives.
Last year, Amps began playing in South Florida with the T&T Group. Led by President Larry Hall, the group comprised retired and active military personnel, band leaders, educators, consultants and administrators. Among them was Lt. Colonel Ivery Taylor, who is currently stationed in Iraq.

A member of Alpha Phi Alpha Fraternity, LTC Taylor soon learned that nine other soldiers in the 13th Sustainment Command (Expeditionary) were Alphas as well. To pull them all together, ILT Christopher Mark sent an email inviting all the Alphas to meet for Sunday Dinner. According to LTC Taylor, “Hard times make one search for common ground. We began to search each other out and as a result, we Alphas now meet weekly for dinner and fellowship. It helps us deal with the rigors of family separation and war.”
After being introduced to Lt. Colonel Taylor via email, I soon began corresponding with the other soldiers of the 13th Sustainment Command while working on this story, which initially focused on the fraternity. After weeks of “talking” via email, I gained a new level of respect and admiration for, not only these proud members of Alpha Phi Alpha Fraternity, but for all our soldiers serving in Iraq. Communicating with these men, some of whom are not much older than my own sons, shifted my view of them from “soldiers on a mission” to sons, brodiers, nephews, dads and cousins. I enjoyed my many emails with them, and in fact, rushed to check my email daily, hoping for a new message from one of them. I have enjoyed working on this story, and am extremely proud of each and every one of my new friends - the very brave Alphas in Iraq.

BC: What made you the join the military?

Lieutenant Colonel Shan Kevin Bagby (Newark, N. J.)

My father and older brodiers served, so it seemed like a natural choice growing up. Finances were very tight, so I attended college on an Army ROTC scholarship. After graduation from college and receiving a commission, I served in the Reserves while completing dental school and residency. I volunteered for active duty service because I found within the Army a diverse group of honest, hard working and like-minded people focused on a common purpose. That really appealed to me and still does. I have enjoyed my time in the Army immensely.

Lieutenant Tracy Brown (Monticello, Hiss)

I joined the military after college for the money, and because it was a fast way for me to find employment. Now, however, it is not about the money; I do it because I want my family and all Americans to have freedom.

BC: In addition to Sunday Dinner, what other activities do you do as a group?

1LT Christopher Mark (New Orleans, La)

I have spearheaded an event called “Just Came Here to Chill.” The event consists of open mic poetry, jazz, and old school R&B. The purpose is to give soldiers a chance to relax and talk about whatever is on their minds. I find this helps the soldiers deal with the stresses of war.

BC: How do you feel about the mission in Iraq?

Lieutenant Colonel Ivery J. Taylor (Waco, Texas)

I am a soldier with a mission and failure does not enter my mind. We are bringing freedom to a country that has not experienced it in quite a while. There are so many positive things the soldiers do such as construction projects, provide medical and dental care to people who really need it, and assist in establishing the infrastructure. The Iraqi people deserve to have the chance to become a self-sustaining government. It will not be the U.S. military that stops the violence; it will be the Iraqis diemselves. However, we will do whatever we can to prevent Civil War. The irony of the matter is America had a Civil War so we know first-hand how devastating it can be. Mission Impossible-No; Difficult-Yes; Worth It-Hell Yes.

BC: What lessons have you learned in Iraq that you will carry with you always?

Lieutenant Colonel Shan Kevin Bagby (Newark, N. J.)

1) You don’t need a lot to be content, and sometimes the less “stuff” you have, the happier you are - so keep life simple.

2) We in the West consume more than our fair share of natural resources and the global effect is politically, socially and economically tangible.

To the Editor:

Dr. Mannino and colleagues in their recent article reported that the most rapid decline in lung function in the Atherosclerosis Risk in Communities (ARIC) study was associated with increased risk of death (1). The rate of change was defined as the percentage change in FEV1 at 3 years from baseline in participants aged 44 to 66 years at baseline. Overall, participants with the most rapid declining lung function had a modestly increased risk of death. However, the method of defining rapid declining lung function introduces a bias that puts to question some of the conclusions. This can be demonstrated as follows.
For healthy males, standing height 178 cm, predicted FEV^sub 1^ was computed at an average age for the age groups quoted in Mannino and coworkers’ article (Table 1) (2). In Mannino’s approach, FEV^sub 1^ should decline proportionately; thus, a 0.75% annualized change from baseline at age 47 years comes to 30 ml, approximating cross-sectional findings in their healthy reference populations. In the oldest person, a 0.75% annualized change is only 26.4 ml. Assuming a uniform rate of change in perfectly healthy subjects of 30 ml/year (last column of Table 1), expressing change as a percentage of the initial value inflates the observed percentage rates of decline in older subjects. This is exacerbated by the fact that longitudinal studies (3, 4) (and references therein) have revealed an age-related accelerating decline in FEV^sub 1^.
This implies an age-related bias in the annualized rate of change of FEV^sub 1^ (i.e., the number of elderly persons will be artificially increased in the group of rapid decliners); using age as a covariate does not resolve this bias. If the standard deviation of repeated measurements in the same person does not decline across age categories, this will exacerbate the problem because expressing change as a function of initial value is then influenced by regression to the mean (5). One wonders whether an approach like that of Burrows and colleagues (3), standardizing FEV^sub 1 ^for height cubed, or that of Chinn and coworkers (6), standardizing FEV^sub 1^ for height squared, would not be more appropriate. An advantage of these approaches is that an apparently accelerated decline in FEV^sub 1^ due to the decline in standing height with age is thus taken into account, removing another source of age (and height)-related bias. Even so, a 3-year interval is too short to accurately estimate longitudinal decline (3).

Conflict of Interest Statement: P.H.Q. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. J.P.S. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. M.R.M. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. G.R. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript.

Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related, high-grade, round-cell tumors with a neuroectodermal phenotype. These tumors are histologically considered on a morphologic spectrum, and they express similar genetic alterations. ES usually develops in bone and is more undifferentiated, while PNET tends to involve soft tissue and demonstrates more pronounced neuroendocrine features.

ES/PNETs are more common in children and young adults, with about 20% of ES/PNET patients developing head and neck disease. There is a minor male preponderance. These tumors are often polypoid, and they can become quite sizeable (up to 6 cm). They are often associated with bone erosion with ulceration and bleeding. In view of the anatomic confines of head and neck sites, tumors in this region are usually much smaller at presentation than those at other anatomic sites.
Histologically, ES/PNETs are made up of diffuse, densely cellular sheets of uniform, small to medium-sized round cells with scant vacuolated cytoplasm (figure 1). The nuclei are round with a fine, delicate to coarse chromatin distribution and small nucleoli (figure 2). Mitotic figures are common. Coagulative necrosis is frequently identified. Occasionally there is a greater degree of nuclear pleomorphism with a rosette formation. Conceptually, the tumor is classified as a small, round, blue-cell neoplasm, which requires the application of special studies to confirm the diagnosis. The tumor cells contain glycogen, which is highlighted with a periodic acid-Schiff (PAS) stain (figure 2). CD99 and vimentin are almost always expressed in ES/PNET, while neuron-specific enolase and synaptophysin are expressed less often. FLI-1 (a portion of the gene fusion product of EWS/FLI-1) can be detected by immunohistochemistry, although the characteristic chromosomal translocations at t(11;22) (q24;q12) or t(21;22) (q22;q12) can be identified by polymerase chain reaction or fluorescent in situ hybridization.
The differential diagnosis includes other small, round-cell tumors, such as lymphoma, rhabdomyosarcoma, olfactory neuroblastoma, melanoma, sinonasal undifferentiated carcinoma, and pituitary adenoma. Different clinical presentations, patterns of growth, immunohistochemistry findings, and molecular studies allow for separation.

Tumor stage is one of the most important considerations in patients with this highly aggressive neoplasm. ES/PNET is managed with multimodal therapy. Patients with sinonasal tract lesions and those with the EWS/FLI-1 fusion tend to have a better prognosis than do patients with thoraco-abdominal lesions and those without the fusion.

Suggested reading

Toda T, Atari E, Sadi AM, et al. Primitive neuroectodermal tumor in sinonasal region. Auris Nasus Larynx 1999;26:83-90.

Wenig BM, Dulguerow P, Kapadia SB, et al. Neuroectodermal tumours. In: Barnes EL, Michael L, eds. Pathology and Genetics of Tumours of the Head and Neck. Kliehues P, Sobin LH, series eds. World Health Organization Classification of Tumours.