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Lindblom diagnosed with cancer. Out for the season


albertaflyer

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Oh my God.... What a bummer.  When something like this happens, hockey is the last thing that comes to mind, obviously.  His focus now will be full recovery and being able to enjoy the rest of his life.  Ewing's sarcoma, while rare,  affects largely teenagers and young adults.  The good news is that it is highly curable, especially if caught early.  I wish him the best.

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I received the text with this terrible news a half an hour ago.  I see people are on it already.  I will include him in my nightly prayers.  My heart goes out to the Flyers organization and to him.  I hope he pulls through this terrible disease that is ruining lives all over the world daily. 

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I read that it’s a very rare form of cancer and it’s very aggressive. This poor kid, I hope he somehow beats it. Wow is there another team with the bad luck of the Flyers. Lindbergh, Dupree, Trytishny and now Lindblom facing a huge battle. I read up on it and it doesn’t sound good. Terrible news.

Edited by RonJeremy
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I dont know how early they caught this...but I can make a somewhat educated guess and say if he was not a pro hockey player and subjected to extensive testing...probably would not have found it this early...how many young men in their 20's get tested for something like this? I'm betting not many...so thank the good Lord he IS a pro hockey player...huh?

.

 

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1 hour ago, jammer2 said:

how many young men in their 20's get tested for something like this? I'm betting not many...

 

 

The problem with cancer in general is that even tests won't always signal a problem.  It's usually symptoms that make one suspect that something is very wrong.  And when they *do* test, it often happens to be too late.  Ewing sarcoma is aggressive, but is one of the most curable forms of cancer as long as it's not spread (in which case the survival rate is significantly lower).  Let's hope and pray it's not spread and he beats it.

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https://www.stjude.org/disease/ewing-sarcoma.html

 

From the Primary source I use at work ...not sure if the link will work outside of my institution ....

 

https://www.uptodate.com/contents/clinical-presentation-staging-and-prognostic-factors-of-the-ewing-sarcoma-family-of-tumors?search=ewing sarcoma adult&source=search_result&selectedTitle=1~96&usage_type=default&display_rank=1

 

CLINICAL PRESENTATION

Primary sites — The Ewing sarcoma family of tumors (EFT) most often arise in the long bones of the extremities (predominantly the femur, but also the tibia, fibula, and humerus) and the bones of the pelvis. The spine, hands, and feet are affected considerably less often [8,9]. In a compilation of data from 975 patients from the European Intergroup Cooperative Ewing Sarcoma Studies (EI-CESS) trials, the distribution of primary sites was as follows [9]:

Axial skeleton – 54 percent (pelvis 25 percent, ribs 12 percent, spine 8 percent, scapula 3.8 percent, skull 3.8 percent, and clavicle 1.2 percent)

 

Appendicular skeleton – 42 percent (femur 16.4 percent, fibula 6.7 percent, tibia 7.6 percent, humerus 4.8 percent, foot 2.4 percent, radius 1.9 percent, and hand 1.2 percent)

 

Other bones – 0.7 percent

 

A minority of Ewing sarcomas (ES) arise in soft tissue. Compared with undifferentiated ES of bone, patients with extraosseous ES (EES) are more frequently older, more likely to be female, and arise more often within the axial, rather than the appendicular, skeleton [8,10-12].

 

Signs and symptoms — Patients with EFT typically present with localized pain or swelling of a few weeks' or months' duration [13-15]. Trauma, often minor, may be the initiating event that calls attention to the lesion. The pain may be mild at first but intensifies fairly rapidly; it may be aggravated by exercise and is often worse at night. A distinct soft tissue mass can sometimes be appreciated. When present, it is usually firmly attached to the bone and moderately to markedly tender to palpation [16,17]. Swelling of the affected limb with erythema over the mass is not uncommon.

Patients with juxta-articular lesions may present with loss of joint motion, while lesions involving the ribs can be associated with direct pleural extension and large extraosseous masses [18]. When the spine or sacrum is involved, nerve root irritation or compression can result in back pain, radiculopathy, or symptoms of spinal cord compression (eg, weakness or loss of bowel and/or bladder control). (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression".)

Constitutional symptoms or signs, such as fever, fatigue, weight loss, or anemia, are present in approximately 10 to 20 percent of patients at presentation [13]. Fever is related to cytokines produced by the tumor cells and, along with other systemic symptoms, is associated with advanced disease.

 

Approximately 80 percent of patients present with clinically localized disease, although as noted previously, subclinical metastatic disease is presumed to be present in nearly all. Overt metastases may become evident within weeks to months in the absence of effective therapy. The significance of this lies in the frequent delay between the onset of symptoms and diagnosis, which in one report averaged over nine months [15,19].

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PROGNOSTIC FACTORSSeveral clinical and biologic characteristics can assist in defining prognosis and directing the intensity of therapy [49]. These include the presence or absence of metastases, the primary tumor location and size, age, response to therapy, and the presence of certain chromosomal translocations.

Disease extent — The key prognostic factor in Ewing sarcoma is the presence or absence of metastases. Approximate five-year survival rates for patients with localized disease are 70 percent, while they average 33 percent for those who have overt metastases at diagnosis. (See "Treatment of the Ewing sarcoma family of tumors", section on 'Treatment for localized disease' and "Treatment of the Ewing sarcoma family of tumors", section on 'Treatment for metastatic disease'.)

The location of distant disease also impacts outcomes. In the European Intergroup experience, the five-year relapse-free survival rates for patients with localized and metastatic disease at presentation were 55 and 21 percent, respectively [9]. Patients with bone and lung metastases fared significantly worse than those with bone metastases alone, who in turn, fared worse than those with isolated lung metastases. Approximately 30 percent of patients with metastases limited to the lungs will survive five years, as compared with only 10 percent of those with bone or bone marrow involvement.

Patients with limited pulmonary metastases who are candidates for resection may have a reasonable opportunity for cure. (See "Treatment of the Ewing sarcoma family of tumors", section on 'Pulmonary metastases'.)

 

Tumor site and size — For patients presenting with localized disease, those with axial primary tumors (ie, pelvis, rib, spine, scapula, skull, clavicle, sternum) have a worse treatment outcome than those with extremity lesions [9,50]. In one series, the five-year relapse-free survival rates were 40 versus 61 percent [9]. In addition, patients with small primary tumors (<100 mL) fare better than those with larger tumors [9,51]. Fever, anemia, and elevated serum lactate dehydrogenase (LDH) all correlate with a greater volume of disease and a poorer prognosis [9,20,46,47].

 

The poorer prognosis of large primary tumors, and those involving the pelvis and spine, is at least partly attributable to the difficulty in achieving wide negative resection margins and to higher rates of local failure after radiation therapy for large lesions. (See "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management", section on 'Non-extremity lesions' and "Radiation therapy for Ewing sarcoma family of tumors".)

 

Extraosseous, as compared with osseous, origin does not have an adverse influence on survival [11,52,53]. In fact, tumors that arise in skin or subcutaneous sites have a generally favorable prognosis [53-55].

Response to therapy — Patients with apparently localized disease have only a 10 to 20 percent likelihood of cure if treated with surgery or radiation therapy alone; this is improved dramatically when chemotherapy is added to treatment (see "Treatment of the Ewing sarcoma family of tumors"). Both the completeness of surgical resection and the response to induction therapy are important prognostic factors. Patients who are left with significant amounts of viable tumor in the resected specimen following neoadjuvant chemotherapy do worse than those with minimal or no residual tumor [9,56-60]. These topics are discussed in detail elsewhere. (See "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management", section on 'Neoadjuvant chemotherapy'and "Radiation therapy for Ewing sarcoma family of tumors", section on 'Adjuvant radiation therapy'.)

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SUMMARYEwing sarcoma (ES) and peripheral primitive neuroectodermal tumors (PNETs) comprise the same spectrum of neoplastic diseases known as the Ewing sarcoma family of tumors (EFT), which also includes malignant small-cell tumors of the chest wall (Askin tumor). Because of their similar histologic and immunohistochemical characteristics and shared nonrandom chromosomal translocations, these tumors are considered to be derived from a common cell of origin, although their histogenetic origin is debated. (See 'Introduction' above.)

 

EFT most often arises in the long bones of the extremities (predominantly the femur, but also the tibia, fibula, and humerus) and the bones of the pelvis. The spine, hands, and feet are affected considerably less often. (See 'Primary sites' above.)

 

Patients with EFT typically present with localized pain or swelling of a few weeks' or months' duration. Trauma, often minor, may be the initiating event that calls attention to the lesion. Constitutional symptoms or signs, such as fever, fatigue, weight loss, or anemia, are present in approximately 10 to 20 percent of patients at presentation. (See 'Signs and symptoms' above.)

 

The goals of the initial evaluation are to establish the diagnosis, evaluate local disease extent, and determine the presence and sites of metastatic spread. (See 'Staging evaluation' above.)

The diagnostic work-up is usually initiated with a plain radiograph of the affected area. EFT involving bone typically presents as a poorly marginated destructive lesion with a permeative or "moth-eaten" appearance, most often associated with a soft tissue mass. While computed tomography (CT) scan better delineates the extent of cortical destruction and soft tissue disease, definition of tumor size, local intraosseous and extraosseous extent, and the relationship of the tumor to fascial planes, vessels, nerves, and organs is best achieved by magnetic resonance imaging (MRI). (See 'Radiographic studies' above.)

 

The metastatic work-up includes a CT scan of the chest to evaluate the thorax for metastatic disease and a radionuclide bone scan to evaluate the entire skeleton for the presence of multiple lesions. Positron emission tomography (PET) or PET/CT is increasingly used for the initial staging and response assessment of EFT and may be able to replace radionuclide bone scan for detection of bone metastases in selected patients with lytic disease. Some clinicians advocate bone marrow biopsy (at least unilateral) in all patients to exclude widespread metastatic disease. (See 'Metastatic work-up' above and 'Bone marrow aspirate and biopsy' above.)

 

A biopsy should be carefully planned to obtain adequate diagnostic tissue without compromising a later operation, particularly the opportunity for limb salvage. (See 'Tumor biopsy' above.)

Several clinical and biologic characteristics can assist in defining prognosis and directing the intensity of therapy. These include the presence or absence of metastases, the primary tumor location and size, age, response to therapy, and the presence of certain chromosomal translocations. (See 'Prognostic factors' above.)

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TREATMENT FOR LOCALIZED DISEASE

Chemotherapy — Most modern treatment plans utilize initial (induction or neoadjuvant) chemotherapy followed by local treatment and additional chemotherapy. Reduction of local tumor volume is accomplished in the majority of patients, and this can facilitate resection. This is particularly important with regard to limb-sparing procedures for extremity lesions, but it may also be important for rib, chest wall, and vertebral primaries [31-33]. Initially, chemotherapy was used in the adjuvant setting to control metastatic disease, but it is now administered prior to local therapy (neoadjuvant therapy) to improve local control as well [34]. Since most treatment failures are attributable to systemic metastatic disease, local therapy considerations should never compromise the administration of effective systemic therapy. (See "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management".)

 

Chemotherapy treatment has evolved, largely due to the efforts of several cooperative groups [34]:

In the first Intergroup Ewing sarcoma study (IESS-I), the combination of vincristine, doxorubicin, cyclophosphamide, and dactinomycin (VDCA or VACA) was associated with a significantly better five-year relapse-free survival than vincristine, dactinomycin, and cyclophosphamide (VAC) alone or VAC plus adjuvant bilateral pulmonary irradiation (60 versus 24 versus 44 percent, respectively) [9].

 

Increasing the doxorubicin dose intensity during the early months of therapy further improved response, and in the second Intergroup study (IESS-II), the five-year relapse-free survival rates using intermittent high-dose four-drug therapy improved to 73 percent for non-pelvic lesions [40]. Because of concerns about limiting the dose intensity of doxorubicin in regimens containing dactinomycin [41], dactinomycin was omitted from most trials thereafter, with no adverse impact on long-term outcome.

 

Adding alternating cycles of ifosfamide and etoposide (I/E) to a vincristine, doxorubicin, and cyclophosphamide(VDC) backbone (VDC/IE) provides further benefit [14,42-44]. In a randomized phase III clinical trial (the IESS-III study), the addition of I/E to VDCA was associated with significantly better five-year relapse-free survival compared with VDCA alone (69 versus 54 percent, respectively) in patients with localized (nonmetastatic) disease but not in those with metastatic EFT or PNET [42].

 

As a result of these data, current standard chemotherapy for EFT in the United States includes VDC/IE [42]. Typically, four to six cycles of chemotherapy are given before local therapy in the absence of disease progression followed by local treatment, and then additional cycles of the same treatments are given postoperatively for a total of 14 to 17 cycles. Relief of pain, decrease in tumor size, fall in lactate dehydrogenase (LDH) level, radiologic improvement, and evidence of necrosis in the resected specimen are all evidence of the antitumor effect of the prescribed regimen.

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I just thought everyone should be as informed as possible .......  hope you don't mind.

 

Full transparency .....  I am not as strong in updated cancer chemotherapy treatments... I just don't do enough of it on a daily basis.

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Just now, Mad Dog said:

@pilldoc

 

Thanks for all this info, Doc.  Just curious is you encountered any patients with Ewing sarcoma in your practice.

 

No .... I primarily work in the surgical unit in the OR.  We have a separate oncology department with pharmacist's who do this all the time.  So no ...I'm sorry I don't have much experience.  I can read the journals and protocols and give an educated opinion, but I have zero interaction with these patients on a daily basis, unless they come to the OR for a mediport or power port placement for their chemo.

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