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A Patient is at the Other End  

“Who’s on call?” Dr. Elliot, chief of ENT surgery, bellowed the question into the resident sign out room. Residents, who were not on call, slinked away while others feigned interest in an acute appendicitis case perched on the stage of the multi-headed microscope. Dan pointed to James who glanced at the wall clock.

Five o’clock on a Thursday afternoon, time for Dr. Elliot’s weekly commando procedure. During the next several hours, Dr. Elliot took a forlorn resident and attending through a plethora of frozen sections with countless orientations of his resected specimens. The patients, eldery men with a long history of alcohol and tobacco use, presented their physicians with a fungating and necrotic tumor that ate away at half of their faces. Dr. Elliot rewarded their lifestyle choices with a radical procedure that may provide a passing chance for survival. Commando was a slang term for a composite resection that removed the tumor as well as half the face. Amongst the residents, it was affectionately referred to as a face-ectomy.

James saw the schedule two weeks ago when it was first posted; he knew he had a date with Dr. Elliot that evening. His only solace was knowing his attending physician was Nomura.

Dr. Elliot motioned to James. “You the resident on call? These are the head CTs of our man, or should I say boy.”

Boy? This was not a typical Elliot case. The CT scans, mounted on the portable radiograph viewing box, tucked away in the corner of the surgical sign out room, revealed an opaque mass situated within the right orbit, that compressed the optic nerve and eroded into the bone.

“This is a 10 year old boy presenting with a month long history of a growing mass in his right orbit. No other medical problems. Family history negative. I’m going to need frozens for diagnosis and then decide where to proceed from there. I’m thinking lymphoma or some small round blue cell tumor. I spoke to Haas when I was coming down the hall, she knows about it.”

“Dr. Haas? Dr. Nomura is on call with me.”

“You’d better check with Haas, she told me she was on call.”

The blood drained from James' head. A quick check with the department secretary confirmed Dr. Elliot’s impression; the two made a last minute switch.

"We'll be starting in a few minutes. I will scope him first and send you a few pieces for diagnosis then depending, upon what you tell me, I'll decide upon the surgery."

Haas looked up from her microscope as James entered. "Did you speak with Dr. Elliot about the case?"

"Yes maam."

"What's your differential?"

"A large soft tissue tumor in the head and neck area of a ten year old boy...a small round blue cell tumor is the highest in the differential."

"And that means what?"

"Uh...it means a lymphoma...a rhabdomyosarcoma....Ewing's sarcoma...a PNET...and um...., a neuroblastoma?"

"And how will we differentiate between these tumors?"

"We can do immunoperoxidase stains, maybe electron microscopy."

"Which immunoperoxidase stains?"

He had reached the point of no return.

"Look it up and tell me before we do the frozen."

James left her office and glanced at the wall clock. Better get some dinner before that frozen comes, James thought. On the way to the cafeteria, some of the internists greeted him in passing.

"I thought pathologists had bankers' hours?"

"Someone has to keep you guys honest."

"Oh yeah, you guys got it so easy. We're up all night admitting patients and you put your patients in the refrigerator."

"Well, I'll be here pretty late tonight with Dr. Elliot's case."

"It's about time you guys earned your keep!"

James waved them by with his right hand; his left hand was clenched. It's a different kind of pressure, James whispered to himself.

"There is a patient at the other end of the slide. Never forget that!"

His father's words resonated. "There is no less pressure in diagnosing a malignancy that will forever change a patient's life than there is in operating all night to remove that malignancy. But always remember that a living breathing patient is awaiting your diagnosis. Do everything possible to make sure you render the correct diagnosis in a timely fashion." A man whose speech was usually delivered with great economy, the length of his father's discourse riveted James' attention and seared his memory. Growing up, he disliked the formality of his father. Separated from him for over three years, he now clung to the memories, an anchor for his sanity.

Returning to his office, dinner tray in hand, James hoped to finish his food before the first frozen section arrived. As he ate, he reviewed a textbook of soft tissue tumors, opened to a review of small round blue cell tumors. The quick perusal refreshed the discussion he had with Dr. Haas. Scanning the pages, he found the information he needed to appease her and jotted some notes. The clock on his desk reminded him of the imminent frozen. As he turned to empty his tray into the trash, he knocked off a recent issue of a surgical pathology journal from his desk. A portion of the title caught his eye. "Another cause of the small round blue cell tumor..." How opportune! James scanned the abstract and flipped through the pages, taking mental notes. The tumor was an extraskeletal mesenchymal chondrosarcoma, a rare tumor that arose in the soft tissue and may occasionally present as an orbital mass. If the tumor was not completely sampled by the surgeon, it may be misdiagnosed. Particularly vexing was the S100 positivity of some of the tumor cells, an immunophenotype that could be shared with other tumors such as a neuroblastoma, Ewing's sarcoma, and PNET, as well as a melanoma. He was about to compare the article's photos to his textbook, when the overhead speaker announced the arrival of the first frozen section.

James and Dr. Haas arrived at the gross room at the same time. Franklin was off during the evening hours so the task of cutting and staining the frozens fell to the pathology resident. James recorded the name of the patient and site of the first frozen. It consisted of an aggregate of red brown tissue; it occupied an area about the size of a quarter on the green surgical cloth. James measured the tissue and lightly touched a slide to the surface of the tissue, for a touch prep cytology specimen. The rest of the tissue was utilized for frozen section.

"Bring the frozen to my office when you are finished. I'll review the touch prep."

James nodded and within a few minutes, he completed the staining of the frozen section slides and placed it next to the Haas' microscope.

"What do you think?"

The tissue sections were diffusely blue, a sign of crush artifact, usually an indication that either he roughly mishandled the specimen, or the tumor was rapidly growing, leading to fragility of the cells. James was sure it was the latter but hoped Haas would agree.

"Looks like a small round blue cell tumor, lots of crush artifact."

"So what's your diagnosis?"

"I don't think we can make a diagnosis...yet. We should let Dr. Elliot know the tissue section is adequate for diagnosis and ask him to send more so we can submit some additional tissue for cytogenetics."

Haas looked up from the dual-headed microscope and nodded, a slight smile cracked her lips. "Good James." Haas quickly dialed the operating room. "Stan? Irene Haas here. Yeah, it's a small round blue cell tumor...no...can't make anything more of it...yup...probably by the end of tomorrow. Send some more for cytogenetics. I'll send my resident in."

Before the conversation finished, James was halfway to the OR. Dr. Elliot was closing the biopsy site as James entered.

"Looks like you escaped my midnight madness. Here's more tissue, you pathologists like that. You're always complaining I don't give you enough tissue."

James' smile was hidden behind the surgical mask but Dr. Elliot could see his narrowed eyes.

"So when can we get an answer?"

"Probably by the end of tomorrow, we should have some of the IPOX studies back and get an idea."

"So...small round blue cell tumor? Looking at this CT scan, I would bet either a rhabdomyosarcoma or lymphoma. What do you think?" Dr. Elliot motioned to the CT film on the view box.

"The small round blue cell tumor differential also includes a Ewing's sarcoma, a PNET, and a neuroblastoma."

"We staged the patient pre-op, the adrenal glands are clean as is the skeletal survey. Hope it turns out to be a lymphoma, he should have a pretty good chance with that diagnosis."

Dr. Elliot's prolific publishing record and engaging teaching style led to promotion to full professor before he turned fourty. Aware of his encyclopedic knowledge, James held his breath and offered up his newly acquired pearl.

"Dr. Elliot, there is another tumor that might be considered, an extraskeletal mesenchymal chondrosarcoma." Dr. Elliot looked up and digested the comment, as if deciding whether he should return the bottle from a freshly poured glass of wine.

"Interesting...I have never had a case but one of my colleagues at Children's Hospital removed one from the orbit of a teenage girl. You know...the clinical presentation may fit with that diagnosis. Can you reliably distinguish it from the other tumors?"

"We can!" James' brash answer surprised himself.

"Then do it! This tumor is placing a lot of pressure on the optic nerve. Call me when you have the preliminary findings. "

"Thanks Dr. Elliot." Leaving the OR with renewed vigor, he could spend the entire evening doing frozen sections if it meant this kind of professional comraderie. Dr. Haas met James outside of the grossing room as he entered.

"James, make sure you instruct the histology lab to cut our case first. Bring the slides to me as soon as you get them so we can decide which IPOX stains to order."

"I'll take care of it, Dr. Haas. Good night."

What a night! Haas and Elliot were on his side! The gross room was cleaned and returned to order but his night had just begun. James returned to his office to completely review the article, taking notes for the suggested immunoperoxidase workup. Another hour passed before James left. Only five hours separated a quick return to his office at 6:30AM.

The completed slides were neatly placed on his desk. The frozen section, performed only a few hours earlier, was the first to be reviewed. Good, it correlates, James thought to himself. As he eyed the additional slides of the tumor, James was pleased the diagnosis was still the same small round blue cell tumor, nothing to add at this point. Looks like a case for ipox to solve, James thought.

Dr. Haas arrived at 7AM, stopping by James' office before she reached her own.

"Good, you have the case. Let's review it in my office in 5 minutes. Were we correct?"

"Yes, maam. I think we will need to do an immunoperoxidase workup."

The slides in her office, Haas quickly reviewed the slides. "Agreed, " Without looking up from the microscope, she asked, "What would you order?"

"I would start with a cytokeratin, desmin, CD99, LCA, WT1, and S100. I would cut additional blank slides in case we need to do a further workup."

"That's a good start." Haas smiled and took a sip of her coffee. "You reviewed the case like I asked you."

James mustered a smile. "I'll order the stains right away. We should have it back by this afternoon."

"Check on the cytogenetics, make sure they have the tissue."

James ordered the stains and checked on the status of the cell culture. I can do this, he thought. I can do this!

The day's cases trickled in from the histology department, brought to him by the chief histotechnologist, Wilma Rousseau.

"Well, Dr. Deetan, I see you've been working overtime!"

"Good morning, Wilma. It wasn't the usual all-nighter with Dr. Elliot. I was home by midnight. Thanks for getting this case out so early. Think we can get the immuno stains by this afternoon?"

"Only for you Dr. Deetan!" A playful blown kiss alighted on the blushing cheek of James. Wilma's light Cajun accent delighted him. Growing up in the Philippines, he had never heard the rhythmic dialect, until their first meeting. Since arriving from New Orleans, Wilma distinguished herself as a skilled histotechnologist and was soon promoted to chief histotechnologist. She was adept at managing others as she was in preparing tissue sections. Thirty three years of age, she appeared much younger with a milky-white complexion framed by auburn hair. Casual flirtation was her middle name but only at a distance. She drew men in with a playful look, but was quick to define her territory when they entered her space.

"Hey, I thought I was your honey!" Carlos playfully touched her shoulder from behind.

"Down boy! I'm out of your league!"

"And what league is that?"

"I need a responsible man...a gentleman, who knows how to respect a woman. Right, Dr. Deetan?"

A quick glance darted between Wilma and Carlos led him to conclude a departure was his best answer. "I need to sign out my cases."

"That is what a gentleman does! Thank you Dr. Deetan." Carlos threw his hands in the air and winked at Wilma.

James organized his cases for signout with Dr. Haas as the lunch hour passed. As he was about to leave his office, Wilma arrived with the immunoperoxidase slides he ordered that morning.

"As promised!"

"Wow! You've never given me the slides this quickly."

"I know, " Wilma whispered. "I told you, only for a gentleman."

"I'll try my best...uh, thanks..." With a bat of her eyes, Wilma disappeared. With no time to review the slides, James took them to Dr. Haas' office.

"Dr. Haas? Wilma just handed me the ipox slides on our case. Would you like to review them first?"

"Well! I'm glad she's finally getting these stains to us before the end of the day, when we can do something about them!"

"Yes, maam." Sitting opposite Haas, he placed the flat of slides next to the microscope.

"Hmmm, just as I thought!" Haas whipped through the brown colored immunoperoxidase slides. "It's definitely not a lymphoma or rhabdomyosarcoma. CD99 and S100 positive-up, this is probably a PNET, in the spectrum of Ewing's sarcoma. Why don't you call Elliot and let him know."

"I heard my name!" On cue, Dr. Elliot appeared at the door. "So, you have an answer for me?"

"Stan...looks like a PNET."

"Really?" Dr. Elliot's face wagered a mild surprise. "What happened to the extraskeletal mesenchymal chondrosarcoma?"

"The what?" Dr. Haas' face turned red as she turned to James, his face now drained of color.

"What you told me about, " Dr. Elliot pointed to James. "You said it would be no problem to differentiate a chondrosarcoma from the other small round blue cell tumors. I contacted my colleague over at Children's and went over my case with him. It's an almost identical clinical presentation with the case he had."

Dr. Haas focused on the slide, her monotone voice unwavering. "That is an interesting differential diagnosis, extraordinarily rare, but I will certainly consider it."

James hurried to stop the hemorrhaging. "Dr. Haas, before I came in, I saw an article about these orbital chondrosarcomas. If they are not adequately sampled, they may be misdiagnosed as small round blue cell tumors. I made a photocopy of the article and was going to give it to you-"

"Yes, I saw that article last month. When you have more experience, you will know when to hunt for zebras instead of ruling out the obvious."

"So, the special stains ruled out a chondrosarcoma, eh? Well, James, you were right! You can nail the diagnosis. You've got a real smart resident there, Irene."

"Yes...real smart." Haas refused to lift her eyes from the microscope. "Listen Stan, James is inexperienced. The staining pattern can be supportive of a diagnosis of a chondrosarcoma but that would be an extraordinary case. I know the article talks about this tumor in this setting, but you know when they write these things, it's always to report some rare tumor in a rare clinical setting, just padding their bibliography."

"Perhaps...but are you absolutely sure this is not a chondrosarcoma?"

Haas pushed herself away from the microscope. "There is less than a 1% chance this is a mesenchymal chondrosarcoma, but no...I cannot absolutely rule it out. We have some tissue cooking in cytogenetics. We can look for the translocation that is present in PNET just to make sure."

"When can I have the results?"

"In a few days."

"Look, Irene, would you mind running this case by Pittman at Children's?"

Haas' face flushed. "You're telling me how to do my job?"

"Just do it!" Elliot headed for the door. "Call me when you have Pittman's answer."

James attempted another bandage. "Dr. Haas, I'm sorry about this misunderstanding. Last night when I left your office and returned to the OR to pick up the additional tissue, Dr. Elliot and I-"

"The next time you decide to make me look like a fool," her voice rose in volume with each word, "is the last time you will be a resident in this program."

James re-organized the paperwork but Haas turned away from the desk. "We're done! Finish it off with Rosenthal!"

James' world imploded. He gathered his cases and sought out Dan in the adjacent office who listened to the entire conversation through the thin wall. Dan motioned to James to sit down.

"She'll show Pittman the case this afternoon. I'm sure it's a PNET. He's one of the best for diagnosing pediatric soft tissue tumors."

James shook his head. "For my sake, I hope it isn't."

"Look, you did what you thought was best, it just didn't come out the way it should have. It was just bad timing. You won the battle but lost the war."

"I don't care. I don't care about any of this. Everything happened so quickly, I didn't have a chance to explain."

Dan smiled. "I don't envy your position right now, but you know something, that was a great call. I did read that article and the differential did cross my mind when I overhead the immunoperoxidase staining pattern. Didn't know you had time to read the journals!"

"I don't! It was an accident, the journal fell open to the article when I knocked it over."

"There are no accidents, it was meant to be!" Dan declared as he took the flats of slides from James. It was 5PM before James and Dan completed the cases. James returned to his office to find a note to call Dr. Elliot. Now what, he muttered to himself, as he punched the extension.

"James! So, you were right!"

"I'm sorry, Dr. Elliot, right about what?"

"The chondrosarcoma! Haas called me a few minutes ago. She reviewed the case with Pittman who said it was a classic mesenchymal chondrosarcoma. Apparently there were a few additional slides that Haas had not reviewed. It had the cartilage which nailed the diagnosis."

James felt even worse. "Dr. Elliot, I think we should wait for the cytogenetics studies. If it shows the PNET translocation, we would definitely have to rethink that diagnosis."

"No doubt...no doubt. But Pittman has never been wrong on these kinds of tumors. Call me when you get the final results."

"I will, thanks."

Haas had not yet returned so James surmised she telephoned Dr. Elliot from Children's Hospital. Within a few days, the results of the cytogenetics confirmed no PNET translocation. All of the findings were supportive of the final diagnosis of an extraskeletal mesenchymal chondrosarcoma. Dr. Elliot was determined to give James credit for initially suggesting the diagnosis. News never traveled faster than through the hospital grapevine. Within a few days, the entire pathology department, save one, gave their kudos to James. Haas had not spoken to him for several days. James later learned of the rearranged attending signout schedule, excluding him for all signouts with her for the next month. A prior family committment was the reason given.

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