CSRF Members' Letters - Ectopic

Code 2076
My name is Jože (29 years old). I live in Ljubljana, capital town of Slovenia (small country in Central Europe; part of European Union). I was diagnosed with a Cushing´s syndrome in March 2003 after a longer period of typical symptoms that presented already a year before that (weight gain, red stretch marks-striae, round face). I had problems with my eye-sight where I still sometimes see some strange patterns which grow and disappear after approx. half an hour. I also noticed acne all over the body (shoulders, back, buttocks, and thighs) which was previously suspected Darriers disease but these disappeared and only occasionally turn up again. Other symptoms that developed later were severe hypokalemia, osteoporosis (lumbar spine cord), mood problems (depression, fear, anxiety, sleeping disorder), pain in the lumbar region, dry skin, swollen ankles, thirst, dry mouth, frequent urination over the night, fast and hard heartbeat, muscle weakness. I also had strong tachycardia 6 times till now.

Since March 2003 I've been hospitalized many times in University Medical Center (Endocrinology) in Ljubljana with purpose to locate the source of excessive ACTH secretion. Next examinations were done in order to localize the ectopic source of ACTH and some of them have been repeated several times: RTG-X rays (chest, abdomen), US-ultrasound (thyroid, abdomen), MRI- magnetic resonance imaging (head, adrenal glands, whole body), CT (thorax, abdomen), whole body scintigraphy with 99m Tc and 111-In octreoctide, whole body catheterization-selective blood sampling (slight increase of ACTH level was present in the sample from vena cava inferior). In December 2003 I also underwent a PET-positron emission tomography scan in Sweden (because there are no PET centers in Slovenia) at Uppsala University Hospital with a tracer 11-C 5-HTP. This study was repeated in January 2006.

I'm taking medications Metopirone (metyrapon) and Oronazol (ketokonazol) to suppress cortisol. Because of hypokalemia and sometimes elevated blood pressure I take Aldactone (spirinolactonum). Most of the symptoms improved but have not totally disappeared with medication and I feel strong side effects of them, still the occult ACTH source remained unrevealed. I attended the London Clinic this year (May 2006) for further investigations when MRI of the pituitary was repeated after 3 years and a 4 mm pituitary microadenoma was reported (left anterolateral aspect of the gland). Comparison with previous MRI scan in Ljubljana dated April 2003 was made and apparently this microadenoma was already visible then and has not changed since. The values of ACTH in my case varies from 15 to 90 pmol/l (ref. value is < 10 pmol/l).

On the basis of first line diagnostics in Slovenia it was excluded that the cause could be pituitary gland but at the London Clinic they pointed out that some things were done shallow:

• MRI-magnetic resonance imaging of the head (pituitary) in April 2003 was reported to be normal
• IPSS (intravenous petrosal sinus sampling after application of CRH) did not show any increase on ACTH levels, but this investigation was performed after I was already on treatment with medications (metyrapone) and the catheters were inserted only to the jugular bulbs and not in to the inferior petrosal sinuses- negative
• 8 DMT (dexamethasone suppression test) showed no significant drop of hormones - negative so my diagnosis was an ectopic secretion of ACTH (suspecting hormonal active tumor somewhere else in the body and not in the head).

Radiologist doctors in MC Ljubljana proposed to make an additional MRI scan and this was done in June 2006. After reviewing all of the images they agreed that there is a microadenoma. It was proposed by doctors (at the London Clinic and MC Ljubljana) to have a pituitary exploration with hope of removing the microadenoma in order to confirm or exclude that the cause of the Cushing´s is originating in the pituitary, which is very likely. I accepted and decided to do so because extensive examinations (in 3 years) did not confirm an ectopic ACTH source and MRI showed pituitary microadenoma. I decided to have the operation at The London Clinic because they are already familiar with my case and such an operation requires an experienced neurosurgeon. Doctors in Slovenia agreed that they don't have much experience for such procedures.

At the moment I'm waiting for approval from doctors and health insurance in Slovenia to provide the funds for the operation in London, UK. Because this operation costs a lot of money the whole process might last months to get an answer which also might be negative. I know this from my own experience in previous diagnostics I had abroad mostly at my own expenses. It took half a year of paperwork and a lot of my personal effort to get the agreement from Slovenian doctors and funds from health insurance just to repeat the PET scan in Sweden. So here is also my personal request. If anyone has any suggestions or can help me gather the needed finances if after a while I don't get the answer from health insurance or they simply refuse to pay for the operation, PLEASE contact me!

Code 2055, Fall 2005 Hi, I am Tim Turner, from Seattle, WA. I had ectopic Cushing’s caused by a lung carcinoma. I was treated by the University of Washington as well as the NIH. I had surgery in December of 2001, and have since been slowly recovering. The photos are with Cushing’s and after the surgery.	With Cushing's	After treatment

Code: 1146 I wanted to thank all of you for your help and information about Cushing's. I was diagnosed with Ectopic Cushing's, Kulchitsky neuroendocrine tumor, carcinoid of the lung. I had been very ill with Cushing's symptoms and the cancer. In Sept. 2000 I had surgery to remove my upper right lobe of the lung and an egg-sized cluster of lymph nodes. That was followed by 7 weeks of radiation. The tumor was producing the ACTH, thus high cortisol. I was like a machine, insomnia, couldn't walk, concentrate, see straight, moon face, beard, loosing hair, face and neck rash, big fat stomach. I gained a lot of weight - up to 178 from 128. Dr. Vance insisted I get an octreotide scan, which would pick up any neuroendocrine type tumor.

Through God, my family, my friends, doctors and nurses and supportive people like you, I'm making it! I am down to 115 lbs. I am cancer free and have had no more Cushing's symptoms so I consider myself a survivor - 3 years and 2 months. I found out a lot about people and life and who really cares about me. Every day is a new gift of love from God. I'm working on publishing my day-to-day journal dealing with Cushing's and lung cancer to help others and give them hope. We're all survivors! After 10 years of misdiagnosis and one illness after another, I'm feeling human again. After 10 years of insomnia, I'm sleeping again. I eat better and I take better care of myself.

Right now, I am having problems with my lower back and leg weakness. I have difficulty sitting, standing, and walking. I continue to have many tests, like MRI's, X-rays, Cat scans, bone density, bone scans, PET scans, blood work and 24 hour urine. I'm still keeping contact with my oncologist, radiologist, and regular physician every few months to test for any changes. I have low potassium and because of pain from bulging discs and a vertebrae out of place in my back, I take Oxycoutin and Hydrocodone. I am on blood pressure medication again. My blood pressure could be elevated because of the pain or lowered potassium. I have been in physical therapy off and on over a year, which helps with the pain and helps strengthen my legs. I tried a chiropractor for a few months but didn't feel like it helped. I would really like to know if anyone else had problems with pain and leg weakness and what they did for it.

Every time I see Lance Armstrong race it inspires me to keep on going. I was walking up and down gorges and waterfalls last summer and tobogganing last winter, thus I consider my leg weakness another temporary thing to overcome. I still need your support and would love to support others. We can share our ups and downs. I saw three grandchildren be born, my son get married, my daughter get her associates degree and I came a great Aunt three times since my surgery. Three years ago I thought I would have been gone by now. Well - I'm still here and still going!!!

Anyone willing to share can write me.

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Code: 1145: Date 1999
I was a 30-year-old successful businessman with a small chain of flower shops in Northern Virginia. The very first symptom I noticed was a decrease in my ability to maintain my balance. I fell several times over the course of a year. I was involved in a car accident in 1994. The doctor noticed my blood sugar and pressure were
elevated and, ultimately, my primary physician put me on a strict routine of diet and exercise, prescribed 1/2 dozen medications, and insulin injections.

My weight gain increased significantly despite the diet and I craved sweets so much that I would drink pancake syrup out of the bottle! My skin became extremely thin, dry and flaky, like crepe paper. I had no strength in my arms or legs. All my hair fell out from my legs, arms, chest and head. I couldn't concentrate and felt disoriented all the time. I was sensitive to cold and developed huge stretch marks on my stomach and hips. I was depressed and didn't talk to friends and family for weeks at a time.

All these symptoms were side effects from the diabetes, or so my doctor told me! They changed my medication, tested me for cocaine, and referred me to a psychiatrist. I told the HMO administrator that something is seriously wrong and "if you don't find out what it is, I'm going straight to my lawyer's office and discuss the situation with him."

They decided I needed a neurological exam. He felt sure, after a five minute exam, that I had Cushing's and referred me to an endocrinologist. After a CT scan, MRI and 20 urine/blood tests, the cause could not be determined for the source of my Cushing's, and he recommended a petrosal sinus sampling test. My HMO refused to pay until my attorney convinced them otherwise!

The CT scan revealed a "nodule" in my left lung. It had blood vessels running through it and could not be accessed with a bronchoscope. It was very close to my heart and moved in conjunction with my heartbeat. They presented my options as: we can operate and remove the nodule and if it's the cause, you will be cured. If it's not the cause of the Cushing's, then your body will not heal up properly and your chances for recovery are very slim.

I sold my house, personal possessions, my business and made out my Will. I opted for the surgery. The past 18 months had been very difficult and I wanted it over one way or the other.
The nodule that the doctors dismissed as "nothing" turned out to be an ACTH producing carcinoid that had spread throughout my left lung and lymph nodes. I was in intensive care on a respirator and the doctors told my family that I did not handle the surgery well because my body was so weak. Despite the doctors telling me how sick I was, I felt like a new person. It was ironic that for 18 months I've been telling them I was sick and they didn't believe me. Now they're telling me I'm sick and I don't believe them!

I discharged myself after 21 days (against the doctor's wishes) and underwent 8 weeks of daily radiation therapy. At the end of therapy, I flushed all my medications down the toilet and went back to work.

Three annual nuclear scans have been cancer free! I lost 50 pounds, my hair grew back and I've regained a good portion of my strength. I don't take any more pills and my blood sugar and blood pressure are normal! I bought a new house in Florida and have basically started a new life. I still suffer from some fatigue and I still have my stretch marks. But these are MINOR issues compared to what I went through with Cushing's Syndrome.

It can be beaten though, I beat it, and if I can beat it, then ANYONE can!! If there is anything I can do to help someone else get through this terrible affliction, please contact me. I have a great deal of free time on my hands (thanks to many years of hard work) and I'm available during the day or evenings on most days.

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Pituitary Tumor
Adrenal Tumor
Bilateral Adrenal Hyperplasia
Ectopic
Under 18 at Diagnosis
Male Members
Steroid Medications and Undiagnosed

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