Paraneoplastic syndrome due to angiomatoid fibrous histiocytoma: a known presentation of an uncommon diagnosis in a rare site and age

Patient’s perspective

THE APPLE AND DR OHR

When I was sick and lay a-bed,

I had two pillows at my head,

And all my toys beside me lay

To keep me happy all the day.

I was the giant great and still

That sits upon the pillow-hill,

And sees before him, dale and plain,

The pleasant land of Counterpane.

—Robert Louis Stevenson

When I was sick and lay a-bed, most of the time I didn’t feel like reading. It was inexplicable: a distinct aversion to the written word, especially if it seemed intent upon inspiring. I had no inclination or energy to look inward, or upward. Answers didn’t interest me, nor questions. I didn’t feel like thinking.

I didn’t want visitors, either, but at least that could be attributed to something physical: my physical weakness, at the time still undiagnosed.

For I was so tired. I slept by day as well as by night, and when not sleeping, I lay in bed. Yet I wasn’t bored, that was the strange thing. I would just lie there, looking out the window. That whole summer, the tree out my window stretched out to me generously with open arms, a friend with great, long limbs adorned with green leaves and birds, keeping me company all the livelong days.

My ESR numbers had been climbing steadily for several years, and I knew what those initials stood for. For when I was growing up, my father, the writer Norman Cousins, had come down with a crippling, painful, never-conclusively diagnosed illness characterized by an elevated sedimentation rate. I’d wonder, mildly curious, if his inflammatory response was some sort of genetic antecedent to mine.

At one point during my husband’s search for answers, he brought me to see a highly acclaimed physician renowned for his prowess as a diagnostician. The waiting list was such that the only available appointment was after 11:30 at night, in his home. The doctor examined my medical history, spent a half-hour or so asking many questions, and then, with knitted brows, he quietly declared: “You know, we doctors like to think that we understand what these numbers mean. But we don’t. It could mean so many different things.” He offered to give me a prescription for painkillers, and another prescription for steroids, to bring down my inflammation. But my parents had never been in favor of medication whose purpose is to suppress symptoms rather than cure them, and I followed their example.

The doctor didn’t argue. He apologized for his inability to provide a diagnosis, made note of the fact that apart from inflammation, I appeared to be in good health, and said it was possible that whatever it was that ailed me might simply subside on its own, without medical intervention.

*

My conscience was burdened by the time and attention my family was devoting to this whole business. My children—busy adults now with young families of their own—were doing anything, everything, that they could to help. Taking turns accompanying my husband and me to this doctor and that one, that one and this one. They went together each week to the Kotel to doven for my recovery, month after month, and I was so grateful. But my gratitude was heavy to bear, because in my heart of hearts, I suspected that nothing was really wrong…nothing, at least, that a good dose of my father’s brand of willpower couldn’t cure.

My tiredness, I thought guiltily, was from depression, and my depression… just laziness in disguise.

Furthermore, although ongoing weight loss was listed as one of my “symptoms,” to my mind it was cause for celebration. As the numbers measuring inflammation kept going up, the numbers on my bathroom scale kept going down…pounds melting away without effort. I weighed now what I’d always dreamed of weighing, starting way back in high school, when I first began to envy that ethereal foreign species of females who have waistlines and no appetites. Nowadays, the mere thought of food repulsed me. So far was I from normal instinctual functioning that I recall sometimes wondering, during that period, what is it that makes people want to eat? It seemed like such an effort to chew and to swallow. Chew and swallow. Chew And swallow.

On the other hand, I took a certain odd, vain satisfaction in the fact that my ESR numbers had skyrocketed even higher than my father’s. The only thing that the hospital in 1964 had been able to offer him was aspirin, as many as 30 a day. After overhearing his doctor confide to someone on staff that he was “afraid they were going to lose Norman,” he and my mother decided to move him into a nearby hotel, rent a hospital bed, arrange for massive doses of Vitamin C to be administered intravenously, and have a home movie screen) set up so that he could watch funny movies. In Anatomy of An Illness (his 1979 book about the physiological impact of laughter, and the full range of positive emotions- hope, faith, love—upon recovery from illness) he would write that 20 minutes of belly laughter had been regularly followed by two hours of pain-free sleep. “A hospital,” he wrote only half-jokingly “is no place for someone who’s sick.”

I myself, though, in my own illness now, many decades later, wasn’t laughing, or hoping, or fighting to keep my spirits up, fighting to survive. If there really was something wrong…so be it.

My bathroom scale, on the other hand, kept giving better and better news, until one morning, at an unguarded moment, I caught sight of myself in the full-length mirror and was taken aback.

This thinness wasn’t beautiful. I was shocked.

I could see my skeleton.

*

I was scheduled for another CAT scan, and blood tests. The wild numbers opened doors. Nurses were kind. If I forgot this or that official document, or came late for an appointment, they’d wave me on in anyway.

I slept. Things were strange. Morning would be afternoon, then I’d wake up and the window by my bed would be shining, and dark. Sometimes the phone would ring, but I was tired. Once upon a time, I’d been a wife, and a mother. I’d been a friend, a sister, an aunt, a grandmother. Once upon a time I’d attended Torah classes, and had loved learning. I’d been interested in things. I couldn’t remember now what that was like. Sometimes I’d take one of my longtime favorites from the bookshelf, but the poetry and fiction that I’d always loved didn’t ring true to me anymore. What did ring true? Holocaust memoirs and histories. Those books were the toys of Stevenson’s poem, but they didn’t keep me happy. Just profoundly preoccupied by the horrendous unreality of the war, and its echoes in the news today.

I’d be startled awake by the dropping of a book from my hands, and would realize I’d dozed off.

One day, with a sort of bemused detachment, I observed something intriguing about my state of mind: if this were a novel, I thought, I’d be the character about whom we’d say: she had lost her will to live.

Oh, I thought, so that’s the story.

I’d arrived at that famous bottom of the barrel, where nothing matters.

There was a certain melodramatic pleasure in defining things this way.

One foot was in the next world.

*

The CAT scan came back and all of a sudden I was put on the fast track to surgery. They’d found a malignant growth. How interesting. I wasn’t faking? It was hard to believe.

The surgeon’s name was Dr. Ohr. Ahhh. In Hebrew, Ohr means light. A nice literary touch.

Next step after the CAT scan was a PET scan, a procedure which required drinking two tall cups of iodine, to determine by way of High Contrast the unidentified growth’s precise location, and position, and if it had spread.

Sitting in the room where a number of subdued strangers were also drinking down the radioactive concoction, each individual in his or her separate enclosure, I read in the PET Information Sheet, among other things, that for 72 hours (three days) after undergoing the scan, the patient was not to be in the presence of children or pregnant women.

I was about to become a radioactive object.

Apparently, it took something of this nature, (talk about high contrast!) to rouse me from my drowsy indifference, for the strange terror of being radioactive--no joke—triggered one the finest moments of my life.

My eyes were lifted up towards the ceiling light, and for a few long shining minutes (my guess is two or three of them) ridiculous vanities and foolishness and insincerities were eliminated from my mind.

*

On the way to the hospital for surgery, I was sitting low in the back seat of a cab (no particular reason to sit up straight) and was gazing up through the taxi window at clouds and sky. No ground. Just clouds…and air….

Clouds….

No ground.

A while later someone said, “Count to ten,” and they were waking me up.

“Everything’s fine,” said Dr. Ohr.

He smiled.

*

Two nights later, at 3 a.m., I felt an unfamiliar, disturbing sensation. There was something I wanted.

My husband, deeply asleep on the chair by my bed, woke in an instant to conduct our search through Hadassah Hospital, and who should we find but one of the Eida Chareidit people who circulate through Israel’s hospital wards throughout the wee hours of the morning, looking for hungry patients such as I. She had a cart full of kosher food, and though I wanted all of it, at that early juncture post-surgery all I could handle was one of her sour apples.

The apple looks good, I thought with wonder. I want it.

Never in my life, not even once, had it ever before occurred to me that appetite isn’t natural. It’s not a naturally-occurring, inevitable phenomenon- a sort of troublesome given, to be taken for granted as one submits to it or resists. It’s a marvelously clever physiological mechanism, purposefully invented to feed living creatures and keep them alive.

*

A few days after the operation, my ESR had plunged all the way down to number 2, at the bottom of the scale.

One sleepless night, back at home not long thereafter, having been kept awake by torment and confusion, unable to see the justification for my return to life--I was lost once again. A more serious case of it, though, this time around, for it hadn’t arisen now from illness but from mundane, regular dailiness. After all the commotion, the fall and the rise and the fall and the rise, why this Rescue Triumphant? A famous line came to mind from one of Shakespeare’s tragedies: the sound and the fury, signifying nothing. For what purpose had my life been given back to me?

The next morning I got a phone call from a friend who has no family in Israel. The friend hinted tentatively that she needed someone to do her a certain favor. Nothing spectacular—just to accompany her to a dentist appointment--because sometimes in the past she reacted to anesthesia and didn’t want to be alone. She’d asked a neighbor, and had called a few other friends, but no one was available. By any chance, she asked, you come? Do you have time?

“Yeah, sure,” I said. “I have time.”

A few hours later, standing with my friend at the bus stop after her root canal, I noticed that beneath my feet was solid ground. My heart expanded and…broke, with joy.

Appetite for Torah learning and chicken soup (with a matzoh ball) reappeared that Shabbos, followed by Apple Crumble (with brown sugar and cinnamon).

Sarah Shapiro

Jerusalem

Learning points

• Paraneoplastic syndrome should be considered in the differential diagnosis and work-up of an unclear systemic inflammatory process.

• Wide excision of the tumour is recommended and resolves all symptoms.

• Paraneoplastic syndrome may be more common with EWSR1-CREB1 translocation.

• A rare tumour in a rare location with relative common symptoms.