The Significance of Expert Opinions in Sarcoma Treatment
Expert opinions play a crucial role in the accurate diagnosis and treatment of sarcomas, rare tumors that can be challenging to identify. Second opinions help ensure correct pathology assessments, with studies showing significant modifications in initial diagnoses upon reevaluation. Central pathologic reviews and expert consultations improve patient management and decision-making, potentially impacting treatment outcomes.
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The Importance of Expert Opinions for Sarcoma Sarcoma Exchange October 3 2020 Charles Forscher MD Co-Medical Director, Sarcoma Program Samuel Oschin Cancer Center Cedars-Sinai Medical Center cedars-sinai.org
Why Bother with a Second Opinion? Not everyone knows everything Is the diagnosis correct? Is the treatment plan correct? Are there other options?
Sarcomas are Rare Tumors A practicing general orthopedic surgeon will see one primary malignant bone tumor in the course of their career The most common diagnosis submitted with a soft tissue sarcoma specimen is Hematoma
Is the Pathology Correct? Given the rarity of sarcomas, it is not unusual for general pathologists to have some difficulty in making a correct diagnosis of sarcoma Southeastern Cancer Study Group Histology Peer Review for Sarcoma 216 patients Concordance in 66% Not a sarcoma 6% Non-concordance 26% Presant CA et al J. Clin Oncol 1986
Role of Central Pathologic Review Rhone-Alpes region of France has a central review process for all newly diagnosed sarcomas Prospective study of newly diagnosed sarcomas for one year 448 patients-366 evaluable 199 full concordance 54% 97 partial concordance 27%- minor change in grade 70 complete discordance 19% major change in grade or histology Lurkin A, et al BMC Cancer2010
Conclusion of French Study Over 45% of first histologic diagnoses were modified at second reading, possibly resulting in different treatment decisions. Systematic second expert opinion improves the quality of diagnosis and possibly the management of patients.
Hemangioendothelioma Rare tumor of soft tissue felt to be of vascular origin May occur as primary tumor in the liver Often mistaken for a carcinoma
Misdiagnosesfor Hepatic Hemangioendothelioma Maklouf HR et al. Cancer 1999
What Difference Does it Make? Hemangioendothelioma of the liver can be successfully treated by liver transplantation Recent review demonstrated 5 year survival for HEHE at 77.2% among 88 transplanted patients Brahmbhatt M et al Transplantation June 2020
Change of Diagnosis Malignant to Benign 70 year old family matriarch comes for a second opinion regarding a presumed malignant tumor of the right shoulder X-rays and scans show a destructive area at the right humerus-felt to be a sarcoma Biopsy not performed Patient is scheduled for an amputation at the outside hospital.
On physical exam swelling noted at right shoulder but no tenderness and patient denies pain History of meningitis as a child X-rays and scans reviewed with bone radiologist and felt to represent neuropathic joint Further work-up revealed syrinx in spine and patient underwent neurosurgery to repair syrinx. No surgery indicated at right shoulder-able to keep her arm
Neuropathic Joint Causes-diabetes, syphilis, chronic alcoholism, syringomyelia, leprosy, congenital insensitivity to pain Results in progressive destruction of bone and soft tissue usually at weight bearing joints Surgery in not usually indicated and there is often impaired wound healing at the site Second opinion saved patient from unnecessary and potentially harmful procedure
Pathology Review Malignant to Malignant 67 year old female artist and writer is seen for an opinion regarding newly diagnosed advanced leiomyosarcoma discovered after work-up for abdominal pain CT scan demonstrates multiple abdominal and pelvic nodules. Seen at several outside hospitals and given a recommendation for chemotherapy with a prediction of poor survival
Past history of removal of benign uterine mass 7 years earlier Strong family history of cancer Seen in consultation Patient declines chemotherapy Pathologist reviews current slides and suggests obtaining slides from prior surgical procedure
Why Does it Matter? Review of both sets of slides confirms endometrial stromal sarcoma in the original specimen and metastatic endometrial stromal sarcoma from the new biopsy ESS is often a low grade sarcoma with an indolent course. It can be responsive to hormonal manipulation Patient was begun on hormone therapy and is still alive 17 years later
Concordant Diagnoses but Different Approaches 29 year old female jewelry designer is seen in second opinion for desmoid tumor of left chest Patient had undergone prior breast augmentation surgery and then developed 9cm mass in that region Biopsy confirmed desmoid fibromatosis
Seen by surgical oncologist who recommended resection of mass along with resection of 3 underlying ribs. Patient had recently become engaged and was distressed at the idea of undergoing such a potentially disfiguring surgery
Seen in second opinion Slides reviewed and diagnosis confirmed Patient offered chemotherapy as option On chemotherapy mass decreased in size Patient got married Mass has continued to decrease, is asymptomatic Patient has never undergone surgery
In the past surgery has been considered the gold standard for management of desmoid tumor and it was a possible correct approach to management of this tumor Newer data suggests chemotherapy and oral tyrosine kinase inhibitors can play a role Further, the French have pioneered the idea of initial watchful waiting for desmoid tumors as some may regress spontaneously
Not Always So Easy 67 year old dentist presents with enlarging left thigh mass. Biopsy positive for high grade sarcoma. Pathology reviewed at 3 other institutions Outside hospital 1 high grade sarcoma Outside hospital 2 not sure if sarcoma Outside hospital 3 benign
At one hospital patient offered amputation, based upon malignant diagnosis Patient elected to try pre-operative chemotherapy for 3 cycles Post-chemo-no progression of mass Surgeon at hospital 3 agreed to do limb-preserving surgery
Limb-sparing resection performed at hospital 3 Pathologist at hospital 3 now changed diagnosis to malignant Pathologist at hospital 2 still was not sure if tumor was benign or malignant Pathologists who originally called tumor malignant, now changed diagnosis to benign
After lengthy discussion, patient elected to hold on further chemotherapy Close observation over last 10 years has demonstrated no local recurrence and no evidence of distant disease
What is Important in Second Opinions The same things that are important in first opinions Careful history with particular attention to any prior surgical procedures Careful physical exam Review of relevant imaging Review of relevant pathology
Where to get a second opinion? Ideally one should seek a second opinion from a different institution than the location rendering the first opinion Often there can be an institutional standard For sarcomas, it is advisable to obtain the second opinion from a high volume sarcoma center
How does one pay for a Second Opinion? Most insurers cover the cost of a second opinion minus the deductibles Medicare will cover second opinions for serious conditions (excludes elective surgery) Organizations such as the Sarcoma Alliance can help defray some of the costs of second opinions
Conclusions Concordant second opinions can be reassuring for patients and their families Non-concordant second opinions may point towards better, more appropriate therapies Better to have the right diagnosis Second opinions may allow patients to avoid unnecessary or possibly harmful procedures Second opinions may suggest alternate therapies, clinical trials Do not feel that your physician will be offended if you seek a second opinion. Most physicians welcome the extra input
If physicians tells you that you do not need a second opinion, thank them, pay their bill and move on
