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Malignant Fibrous Histiocytoma
This is a true clinical story.For obvious privacy reasons, the names of the patients, the doctors and their titles and the institutes have been omitted.
The patient’s story
I always saw myself as a relatively healthy 70 year old. I had high blood pressure, which was fine with a few drugs, and a rash on my thighs which my doctor described as a ‘pre-cancer’ which was obviously under close follow up.
During one of my normal visits to my doctor, I showed him a new rash on my thigh. He looked a bit concerned and sent me for an ultrasound. When I went back with the results he told me that it looked ‘suspicious’ and that it had to be removed.
The operation itself was simple and was done just with local anaesthetic. You can imagine how shocked I was when I received the results and found out that I had a type of muscle cancer called ‘ Malignant fibrous histiocytomas.’ Before I could even take in what was going on I was sent off for a load of different tests to check if the cancer had spread….
The CAT scan was fine, but another scan ,called a PET, showed that the cancer may be in my shoulder and back. I had to have further testing to ‘confirm’ this and in the end they said the cancer hadn’t spread there. At this point I was more than a bit confused but relieved to be told that I didn’t need any other treatment.
Eight months later I was sent to a hospital which specialised in my cancer. Another CT and ultrasound were done, showing that the cancer had reappeared in my thigh and also spread to my lungs. The doctors told me that if I wanted to ‘extend my survival’ I had to have chemo…
For me it was the obvious choice to get all the treatment needed but I was terrified.
The initial four rounds of chemo were done and the cancer got a little smaller, but was still there. The oncologists recommended more chemo. Despite all that I had gone through I wasn’t going to give up now, so I went through two more treatments. This time the cancer didn’t get any smaller.
All I could do was think about the cancer and what could be done. Was there any other chemo that could be tried? Had the cancer been in my back and shoulder all the time? Why hadn’t I had an operation to cut it all out? What about alternative therapy? Where was the best place in the world to treat this disease?
I searched the internet and came across medicalopinion. My doctors had no problem giving me all the files and were happy for me to get another opinion.
The report I received was well written and very thorough. It explained that while the initial operation was the right thing to do, there had been no real need for the chemo as the tumour had been so small. I could have made do with no chemo or may be just radiotherapy.
He also pointed out that the doctors should have taken a sample from the cancer that spread to my lungs so they could be sure what it was before they started further treatment. The chemo that I had gotten was right one and my doctors couldn’t have chosen better, however at this point he thought that more chemo would do me more harm then good.
Another option that he suggested was surgery. It was unlikely to cure me, but it may allow me to live longer. At this point in time any opportunity needs to be seized so I am speaking to my doctors at home about this. There is another option called ‘radiofrequency ablation’ that he said was still in the research stages so I decided not to look into it.
I consider myself quite an open-minded guy, so I thought I should look into the ‘alternative’ treatments as well. medicalopinion also managed to get me in touch with an expert in this. He recommended treatment with ‘Mistletoe’ and said that there has even been research proving its worth. I’ve got the number of the expert and I am going to try it. As I’ve already said, you’ve got to try and do everything you can when you’re in my position.
The following documents are the case history that the patient provided and the experts opinion in response.
Clinical information
Patient’s name: --------
Diagnosis : MALIGNANT FIBROUS HISTIOCYTOMA .
A time of diagnosis the patient was a 70 year old man with a history of hypertension, hyperuricaemia and Cutaneous mycosis confined to the inguinal folds. His medications were Aprovel 300 mg x1/d , Atenolol 50 mg 1xd, Cardura 2 mgx1/d,Kanrenol 100 mgx1/d and Zyloric 300 mgx½ day.
During regular dermatology follow up a subcutaneous lesion on the right proximal thigh was found. An ultrasound of the area was performed in August showing a 2X1 cm formation within the subcutaneous adipose tissue with slightly irregular edges and a dishomogeneous, hypoechogenic, partly vascularised echo structure.
The next week a radical excision was carried out under local anaesthetic at the local hospital. An area of 8.0x3.0cm was removed with a 1.8x1.5x1.2cm lesion within. The histology showed a “ Malignant f ibrous histiocytomas ” with intact edges.
Staging was then carried out; an ECHO showed a normal heart, a CT scan of the thorax and abdomen showed no abnormalities however a PET scan showed increased uptake on the left medial periclavicular region (SUV 10.6) and in one of the last dorsal vertebral somas (SUV 4.3). Objective and instrumental examination was carried out using both local ultrasound and bone scintigraphy showing no pathology .
Further follow up was performed at a university hospital in April.
A repeat ultrasound of the right thigh revealed continuous parenchymal-like formations with regular edges of 1-2cm, forming a cord like structure of questionable pathologic significance, possibly fibromuscular hypertrophy .
Due to the pulmonary spread a course of chemotherapy was recommended with a regimen of adriblastine/e pirubicin and ifosfamide with at least four twenty one day cycles with reassessment every cycle in order to decide on whether to continue or alter the treatment.
The treatment was started in June. The patient was briefly hospitalised to receive the medication during each of the four cycles.A Portacath was inserted before the second cycle. The course of the treatment was uneventful.
A further CT scan was performed in the October showing no progression of the lung nodules in either number or size. There were no signs of pleuropericardial effusion or abdominal involvement.
A further CT scan was recommended for February.
Questions:
- Given that the first-line chemotherapy just carried out did not produce a definitive solution, what are the possible recommended second-line therapies in the event that the next examination shows a relapse of the lung disease?
- Why, in your opinion, has there been no removal and/or surgical investigation of the pulmonary lesions yet?
- Is there a centre of excellence abroad for my illness?
- Are there currently any clinical studies, in
The expert’s opinion
Prof. --------
Senior Oncologist and Consultant in Oncology
Oncology Unit
Affiliated to LRZ Faculty of Medicine
Thank you for the referral and the copy of the medical records for this patient. I had the opportunity to review -------- medical records:
SUMMARY OF THE CASE
This patient is a 70 year old man who first noted a subcutaneous nodule along the posterior of the proximal right thigh. After about 3-6 months of observation an ultrasound was performed. That study revealed 2x1cm hypoechoic subcutaneous nodule. The following week this was resected and a nodule measuring 1.8x1.5x1.2cm was obtained. Pathology revealed malignant fibrous histiocytoma. Resection margins were reportedly negative. An abdominal ultrasound, chest xray and CT scans of the chest, abdomen and pelvis were performed and all studies were reportedly negative. PET scan was performed shortly afterwards and this revealed increased FDG uptake in the left medial periclavicular region (SUV 10.6) and also in a vertebral body (SUV 4.3). Follow up imaging was negative, but it is unclear whether that imaging was performed on both of these areas or only on the vertebral body.
A follow up CT scan was done the six months later ( April), and this study revealed a right lower lobe pulmonary nodule measuring 1.6cm and two other right lung nodules measuring 6mm each. In the left lung there was apparently a very small nodule in a fissure. These were felt to be consistent with metastatic disease. Ultrasound was also performed to evaluate the right thigh resection site, and this study revealed nonspecific changes of uncertain significance. As a result, he was started on chemotherapy treatments with adriamycin (?epirubicin) and ifosphamide. After receiving four treatments (one each 21 days) a repeat chest CT scan was performed. This study revealed a reduction in size of the largest right lower lobe lung nodule from 1.6cm down to 1.3cm. The other nodules also appeared slightly smaller. Two additional cycles of the same chemotherapy were given. Another CT revealed no change in size of any of the nodules. This
IMPRESSIONS
This patient was diagnosed with a subcutaneous malignant fibrous histiocytoma of very small size. This tumor was resected with apparently negative margins. No information is given regarding tumor grade. Staging studies were performed soon after resection. CT scans were unremarkable though no official report is available. The PET scan revealed two areas of increased uptake, but apparently these could not be correlated to findings on CT scan. No additional treatment was provided at that time. In April a follow up CT scan revealed multiple pulmonary nodules with the largest measuring 1.6cm. No biopsy was performed. He was presumptively diagnosed with metastatic disease and treated with four cycles of chemotherapy. From the information provided, it is unclear exactly what he received, but apparently he was treated with ifosphamide and an anthracycline (it is unclear which anthracycline). The dominant nodule only measured 1.3cm on follow up imaging and other nodules apparently also looked to be improved. Again no official report is provided. After two more cycles there was no change.
There is some information not provided, including the grade of the initial tumor, the reports of CT scans and the exact details of the chemotherapy treatment. Despite that, however, some conclusions can be made about this case. The initial treatment with surgery was appropriate, and based on the provided information the extent of surgery was satisfactory. Given the small size and subcutaneous location of this tumor, the risk of subsequent metastatic spread is relatively low. If it were low grade then we would have recommended no further treatment given the small size, though radiation could be considered. If this were a high grade tumor then we would have recommended radiation following surgery. We would not typically treat a small (less than 5cm) or subcutaneous tumor with adjuvant chemotherapy, as there is no proven benefit. Pathology review at a center specializing in sarcoma is important in this case as in every case of soft tissue sarcoma, since there can be confusion regarding the proper diagnosis when specimens are reviewed by pathologists who do not focus on these disease entities.
The pulmonary nodules noted in April, if clearly new compared to the previous CT , would certainly raise suspicion regarding metastatic disease. The subcentimeter nodules are of uncertain significance, since depending on their size they could have been present and just missed on the previous scan. However, a 1.6cm nodule would not have been missed had it been present. It would be important to review the CT films in order to verify the absence of this nodule. Possible courses of action at that point include close observation, biopsy and treatment without biopsy. In this case, I would have attempted biopsy had that been possible. If not, the most acceptable course would be to pursue treatment assuming that the 1.6cm nodule was clearly new. This, of course, assumes that this sarcoma is high grade.
The choice of chemotherapy agents was appropriate (ifosphamide combined with an anthracycline). While we give these agents in a different fashion, and possibly at higher doses, these are both active agents in the treatment of soft tissue sarcoma. Reassessment after four cycles of chemotherapy is also appropriate. Additionally, stopping after six cycles is also appropriate, since anthracyclines can only be dosed to certain levels given their propensity to cause cardiomyopathy. Also, there did not appear to be any additional reduction in size in these lesions after two additional cycles of chemotherapy.
Response assessment in soft tissue sarcoma is often difficult, since lesions can undergo necrosis without significant changes in size. However, the initial reduction in size followed by stabilization does suggest that it is reasonable stop chemotherapy at that point. In our hands, the next step would be surgical resection of all identifiable pulmonary disease. This can be curative in a minority of sarcoma patients, and in this case, given the relatively low volume disease, the surgery could likely be done with a minimum of morbidity. This would also allow for histologic examination and quantification of tumor necrosis.
TREATMENT ALTERNATIVES undefined undefined undefined undefined
The major alternative in this case is resection of pulmonary lesions versus observation alone. I would not recommend further chemotherapy beyond six cycles given issues regarding anthracycline induced cardiomyopathy. Also, I would not recommend changing to another chemotherapy regimen or another form of systemic therapy. If surgical resection were not possible for some reason, then one could consider a local ablative treatment, such as radiofrequency ablation. However, the benefit of this treatment approach would be difficult to predict, since this has not been well studied in the treatment of metastatic soft tissue sarcoma. My recommendation in this case is to proceed with surgical excision of the pulmonary lesions as opposed to observation. I would not recommend additional chemotherapy at this time. Finally, prior to making a decision regarding therapy it would be important to have the initial biopsy slides (and any subsequent specimens) reviewed at a center experienced in the diagnosis and classification of soft tissue sarcoma.
Sincerely yours,
Prof. ----------
The patient also asked for a complementary medicine second opinion:
Complementary medicine second opinion
Thank you for the referral and the copy of the medical records for Mr. ---------
This is an opinion based on the attached clinical information. I have not performed a clinical examination of the patient. This opinion has been requested on behalf of the patient by
Mr. -------- is a 70 years old patient diagnosed with METASTATIC FIBROUS HISTIOCYTOMAS , requiring information on alternative treatment for his conditions.
The best evidence based adjuvant alternative therapy in this case is by a mistletoe extract 1 2
Mistletoe grows as a partial parasite on a variety of trees—particularly pine, apple, plum, poplar, and spruce—across northern Europe and
The name mistletoe is said to derive from the Celtic word for “all-heal.” This correlates with its historical use for everything from nervous complaints to bleeding to tumors.
Active constituents : Several constituents have been shown to contribute to the medicinal action of mistletoe. Most notable are mistletoe lectins (also called viscotoxins), choline derivatives, alkaloids, polypeptides, and polysaccharides. Human pharmacological studies have found that mistletoe extract given by injection stimulates immune system function.3 4 5 6
Some test tube and animal studies suggest that certain mistletoe constituents, including the alkaloids, can also kill cancer cells.7 8
Numerous clinical trials have found that subcutaneous injections of mistletoe extracts can help people with cancer of various organs benefit.9 10 There is no evidence that people with cancer would benefit from receiving mistletoe orally.
Clinical effects of mistletoe :
Fewer side-effects of chemo- and radiotherapy- DNA repair improved in normal cells preferentially Improved resistance to infection
Improvement in warmth organism
Better sleep
Less pain
New initiative in life
Life expectancy : Use of Mistletoe therapy increase life expectancy considerably. A recent study9 indicate that mean survival time in the Mistletoes treated patients was 40% longer than in the control groups.
( Iscador®) 2-3 times a week in the abdomen area.
Telephone +** ** *** *****
--------- M.D.
Medical Director of the complementary medicine department
Drt Health Services
References:
1. Kuttan G. Anticarcinogenic and antimetastatic activity of Iscador.
Anticancer Drugs. 1997 Apr;8 Suppl 1:S15-6.
2. Kuttan G. Prevention of 20-methylcholanthrene-induced sarcoma by a mistletoe extract, Iscador.
Carcinogenesis. 1996 May;17(5):1107-9.
3. Urech K. Mistletoe constituents and cancer therapy. J Anthroposophical Med 1993;10:54–63.
4. Hajto T. Immunomodulatory effects of Iscador: A Viscum album preparation. Oncology 1986;43(suppl 1):51–65.
5. Bocci B. Mistletoe (Viscum album) lectins as cytokine inducers and immunoadjuvant in tumor therapy. A review. J Biol Reg Homeostatic Agents 1993;7:1–6.
6. Bloksma N, Schmiermann P, de Reuver M, et al. Stimulation of humoral and cellular immunity by Viscum preparations. Planta Med 1982;46:221–7.
7. Jurin M, Zarkovic’ N, Hrzenjak M, Ilic’ Z. Antitumorous and immunomodulatory effects of the Viscum album L preparation Isorel. Oncology 1993;50:393–8.
8. Khwaja TA, Dias CB, Pentecost S. Recent studies on the anticancer activities of mistletoe (Viscum album) and its alkaloids. Oncology 1986;43(suppl 1):42–50.
9. Grossarth-Maticek R. Use of Iscador, an extract of European mistletoe (Viscum album), in cancer treatment: prospective nonrandomized and randomized matched-pair studies nested within a cohort study.
Altern Ther Health Med. 2001 May-Jun;7(3):57-66, 68-72, 74-6 passim.
10. Anthony S. Inhibition of lung metastasis by adoptive immunotherapy using Iscador.
Immunol Invest. 1999 Jan;28(1):1-8.