EUH Hunt Conference: What is the prognosis for adenocarcinoma of the lung?

The Bottom Line: There is an overall survival rate of less than 5 years.

  • Stage I: 68-92%
  • Stage II: 53-60%
  • Stage III: 13-36%
  • Stage IV: 0-10%

Reference: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 114774, Non-small cell lung cancer; [updated 2018 May 01, cited 15 May 2018]; [about 31 screens]. Emory access required.

Summary: Five-year survival is only 5-8% in patients with unresectable NSCL cancer. Patients with untreated T1 tumors have a survival rate of 9%. Five-year mortality is higher in men than in women.

5-year survival for patients with lung and bronchus cancer by stage at diagnosis:

  • 18% with disease at all stages
  • 55% with localized disease
  • 28% with regional disease
  • 4% with distant stage disease

A study considering intervention and age found that older age and less intervention is associated with poorer survival:

  • > 80 years: 47% no radiation/surgery, 7.4% 5-year survival
  • 70-79 years: 28% no radiation/surgery, 12.3% 5-year survival
  • < 70 years: 19% no radiation/surgery, 15.5% 5-year survival

Additional Information: DynaMed Plus provides breakdowns by stage, age, and other factors.

For information on the staging of lung cancer, see the IASLC Lung Cancer Staging Project.

Advertisements

EUH Hunt Conference: What is the prognosis for patients with HIV-negative PCNSL?

The Bottom Line: Patients diagnosed with primary central nervous system lymphoma (PCNSL) who are HIV-negative have a higher prognosis compared to those with PCNSL who are HIV-positive. While the median survival range is from 10-20 months for HIV-positive PCNSL patients, there is more variability for HIV-negative patients, survival ranging from 12 to 37 months or longer.

References: Bayraktar, S., Bayraktar, U.D., Ramos, J.C. et al. Primary CNS lymphoma in HIV positive and negative patients: Comparison of clinical characteristics, outcome, and prognostic factors. J Neurooncol. 2011;101(2): 257-265.

DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 115176, Primary central nervous system lymphoma; [updated 2017 Nov 29, cited 2018 April 25]; [about 14 screens].

Fadul CE & Ely P. Schmidek and Sweet’s Operative Nurosurgical Techniques. [Internet]. Philadelphia: Saunders; 2012 Jun. Chapter 12: Management of primary central nervous system lymphomas;[cited 2018 April 25]; p.149-159.

Gerstner ER, Batchelor TT. Primary Central Nervous System LymphomaArch Neurol. 2010;67(3):291–297. doi:10.1001/archneurol.2010.3

Norden, A.D., Drappatz, J., Wen, P.Y. et al. Survival among patients with primary central nervous system lymphoma, 1973-2004. J Neurooncol. 2011;101(3): 487-493.

Westin JR, Konoplev SN, Fayad LE, Medeiros L. Aggressive B-Cell Lymphomas. In: Kantarjian HM, Wolff RA. eds. The MD Anderson Manual of Medical Oncology, 3eNew York, NY: McGraw-Hill. Accessed April 25, 2018.

Summary: Factors such as age and type of treatment patients receive impact their overall survival rate. However, the most important prognostic factor is the status of the patient’s health prior to receiving treatment. Many patients can improve their condition by use of corticosteroids and thus be candidates for intensive chemotherapy-based regimens that are potentially curative (Westin, Konoplev, Fayad, and Medeiros, 2018).

EUH Hunt Conference: Review of Meigs’ Syndrome

Bottom Line: Meigs’ syndrome is also known as Meigs syndrome or Demons-Meigs syndrome. It is characterized by a triad of ascites, pleural effusion, and benign ovarian fibroma, which is a rare triad. “Meigs syndrome…is a diagnosis of exclusion only after ovarian carcinoma is ruled out. The presentation of symptoms and radiographic findings mimics that of metastatic ovarian cancer, creating a significant clinical challenge….The treatment is exploratory laparotomy that includes biopsy of the ovarian mass, lymph node biopsies, biopsy of omentum, and pelvic washings. Unilateral salpingo-oophorectomy is performed in women of reproductive age, whereas total hysterectomy is preferred in postmenopausal women. The prognosis of Meigs syndrome is good and <1% of fibromas progress to fibrosarcoma. The pleural effusion and ascites resolve within a few weeks after tumor resection. We favor the use of chest ultrasound to follow pleural effusion progression, as it is superior to chest x-rays in identifying residual pleural effusion and can detect amounts as small as 3 to 5 mL.” The article reports a case of a patient with recurrent pleural effusions and an ovarian mass. It describes the case in detail and reviews the literature on Meigs syndrome.

Reference:
Riker D, Goba D. Ovarian mass, pleural effusion, and ascites: revisiting Meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan;20(1):48-51.

Kokko Conference: Does TTP (thrombotic thrombocytopenic purpura) result in ARDS (acute respiratory distress syndrome)?

The Bottom Line: Life-threatening ARDS can be associated with TTP. Early recognition of ARDS associated with TTP and timely EP is critical to a favorable outcome.

Summary: Experience suggests that ARDS may occur as a clinical feature of either de novo or secondary TTP; therefore, all patients with ARDS should be evaluated for unrecognized TTP. A high index of suspicion is crucial when a patient with ARDS presents with unexplained thrombocytopenia and MAHA, with or without neurological or mental changes. Because exchange plasmapheresis is a life-saving measure, unexplained thrombocytopenia and MAHA should be sufficient criteria to make the presumptive diagnosis of TTP for the initiation of exchange plasmapheresis.

Reference: Chang JC, Aly ES. Acute respiratory distress syndrome as a major clinical manifestation of thrombotic thrombocytopenic purpuraAm J Med Sci. 2001 Feb;321(2):124-8.

EUH Hunt Conference: What is the treatment for insulinomas?

The Bottom Line: 

  • Diet: small frequent meals throughout the day and in them idle of the night to prevent hypoglycemia (DynaMed Plus, 2017).
  • Medications: Diazoxide is administered to suppress insulin release. For incurable islet cell carcinomas, steptozocin is the best chemotherapeutic agent. Approximately 60% of patients live up to 2 additional years. Toxicity is considerable; steptozocin is not recommended as a routine adjunct to surgical therapy (Doherty, 2014).
  • Surgery:
    • For benign insulinoma surgery is treatment of choice, with a 98% cure rate reported (DyanMed Plus, 2017). Surgical options include: resection of pancreatic neuroendocrine tumors, enucleation if a lesion can be found, segmental resection, distal pancreatectomy, pancreaticoduodenectopy. Laparoscopic surgery may result in shorter hospital stay.
    • For metastatic disease: resection of tumor and metastasis, lymphadenectomy, radiofrequency ablation.

References: Doherty GM. Pancreas. In: Doherty GM. eds. CURRENT Diagnosis & Treatment: Surgery, 14e New York, NY: McGraw-Hill; 2014. http://accesssurgery.mhmedical.com/content.aspx?bookid=1202&sectionid=71521584. Accessed November 08, 2017.

DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 115754, Insulinoma; [updated 2017 May 22, cited 2017 Nov 09]; [about 8 screens]. Emory login required.

Summary: Insulinomas have been reported in all age groups. Approximately 75% are solitary and benign. About 10% are malignant, and metastases are usually evident at the time of diagnosis. The remaining 15% are manifestations of multifocal pancreatic disease, either adenomatosis, nesidioblastosis, or islet cell hyperplasia (Doherty, 2014).

Treatment overview:

  • For locoregional disease
    • manage hypoglycemia using diet, diazoxide, octreotide (but may worsen hypoglycemia in some patients), lanreotide (improves progression-free survival but not overall survival). Other drugs have been used to help control blood sugar.
    • tumor removal: tumor enucleation, subtotal or distal pancreatectomy, pancreaticoduodenectomy
  • For metastatic disease
    • manage hypoglycemia with diet and medication
    • other treatment options include: resection of primary lesions and metastases, ablative therapy (tumor destruction), arterial embolization or chemoembolization, systemic chemotherapy, radiation therapy is under investigation.

(DynaMed Plus, 2017)

EUH Hunt Conference: What are the treatments and outcomes for anti-GABA(B) associated encephalitis with small-cell lung cancer?

The Bottom Line: GABA(B) encephalitis shows typical clinical patterns of afebrile limbic encephalitis and intractable seizures, and is associated with small-cell lung cancer. Immunomodulation and cancer chemotherapy often improve the neurological deficits (Kim et al 2014). Patients with small-cell lung cancer (SCLC) were found to have a shorter survival compared with patients without a tumor (Hoftberger et al, 2013).

References: Hoftberger R, et al. Encephalitis and GABA(B) receptor antibodies. Neurology. 2013 Oct; 81(17):1500-1506. doi:10.1212/WNL.0b013e3182a9585f
Kim TJ, et al. Clinical manifestations and outcomes of the treatment of patients with GABAB encephalitis. Journal of Neuroimmunology. 2014 May; 270(1-2):45-50. doi:10.1016/j.jneuroim.2014.02.011
Summary:  For the treatment of GABA(B) encephalitis, both immunomodulating therapy and cancer treatment in the presence of malignancy are necessary. It has recently been shown that ASE patients with epileptic seizures are often refractory to treatment with standard antiepileptic drugs and, in contrast, may respond well to immunomodulating therapy (Kim et al 2014).

A study done by Hoftberger et al (2013) highlighted four relevant findings: 1) the study confirms that most patients with GABABR antibodies develop LE, 2) two patients had novel forms of symptom presentation, ataxia, and opsoclonus, 3) GABABR antibodies occurred in patients with neurologic dysfunction, with or without SCLC, but were not detected in cancer patients without neurologic symptoms, and 4) analysis of the GABABR antibodies and the repertoire of concurrent autoimmunities showed that the GABABR epitopes are conformational, and the type of concurrent antibodies varies according to the presence or absence of cancer, and may suggest outcome.

The diagnosis of this disorder is important, because in most patients neurologic symptoms respond to immunotherapy regardless of being paraneoplastic or not.

EUH Resident Report: What is antisynthetase syndrome?

The Bottom Line: Antisynthetase syndrome is a clinical condition characterized by arthritis, mechanic’s hand sign, interstitial lung disease (ILD), fever, Raynaud’s phenomenon, and myositis. ILD is the most severe manifestation, occurring in approximately 80% of antisynthetase syndrome patients, and is associated with high morbidity and mortality. Current treatment is based on corticosteroids and a wide number of immunosuppressive drugs, although no clinical trials on this condition have been reported. Prognostic factors in ILD related to antisynthetase syndrome have not been entirely defined.

Summary: A 2012 NEJM case report provides the following clues for diagnosis: The eyelid edema and erythema with pruritus are very characteristic of the heliotrope rash that is associated with dermatomyositis. Malar erythema also occurs and differs from the typical “butterfly rash” of SLE. In SLE, the nasolabial folds are spared from rash, whereas in dermatomyositis, they are not. Other salient features of the antisynthetase syndrome include interstitial lung disease, arthritis, fever, markedly elevated levels of muscle enzymes (creatine kinase, aldolase, and the aminotransferases, with the aspartate aminotransferase level higher than the alanine aminotransferase level), and perhaps most important, a negative ANA test but a positive test for anticytoplasmic autoantibodies.

For treatment, immunosuppressive agents, typically glucocorticoids, with methotrexate or azathioprine as the most common additional agents, are typically used and may result in clinical improvement in patients with inflammatory myositis. Intravenous gamma globulin has been shown to be effective in a controlled study of dermatomyositis.

The patient in the case report continued to have progressive pulmonary disease, fever, myositis, and arthritis and required variable doses of ibuprofen, prednisone, and methotrexate to control the symptoms. The typical rash of the antisynthetase syndrome — mechanic’s hands — eventually developed, and therefore, the patient had all the features of this syndrome.

References: Christopher-Stine , L., et al. (2012). “Case 37-2012.” New England Journal of Medicine 367(22): 2134-2146.

Rojas-Serrano, J., et al. (2015). “Prognostic factors in a cohort of antisynthetase syndrome (ASS): serologic profile is associated with mortality in patients with interstitial lung disease (ILD).” Clinical Rheumatology 34:1563-1569.

Outcomes for near syncope/presyncope and syncope

Bottom line: Patients who present to the emergency department with near syncope and syncope are likely to have similar prevalence, etiology, prognosis, critical interventions, and adverse outcomes. Patients with near syncope are less likely to be admitted.

Sources

Grossman, Shamai A, et al. “Do outcomes of near syncope parallel syncope?” The American journal of emergency medicine 30.1 (2012):203-6.
Study included 244 patients who presented to the emergency department (ED) with near syncope; “follow-up was achieved in 242 (99%). Emergency department hospitalization or 30-day adverse outcomes occurred in 49 (20%) of 244 compared with 68 (23%) of 293 of patients with syncope (P = .40). The most common adverse outcomes/critical interventions were hemorrhage (n = 6), bradydysrhythmia (n = 6), alteration in antidysrhythmics (n = 6), and sepsis (n = 10). Of patients with near syncope, 49% were admitted compared with 69% with syncope (P = .001). Patients with near syncope are as likely those with syncope to experience critical interventions or adverse outcomes; however, near-syncope patients are less likely to be admitted.”

Greve, Yvonne, et al. “The prevalence and prognostic significance of near syncope and syncope: a prospective study of 395 cases in an emergency department (the SPEED study).” Deutsches Ärzteblatt international 111.12 (2014):197-204.
This is a prospective study of 395 patients seen in ED for syncope or near syncope who were followed up at 30 days and 6 months. Those with “near-syncope do not differ to any large extent from patients with syncope with respect to the features studied” (prevalence, etiology, prognosis).

How can the Simplified Pulmonary Embolism Severity Index (sPESI) be used to manage patients presenting with a pulmonary embolism?

The Bottom Line:  The Pulmonary Embolism Severity Index is a validated tool for predicting 30-day mortality in patients presenting at the hospital with PE.  The Simplified PESI (sPESI) predicts 30-day mortality with accuracy similar to the PESI And can assist in deciding whether inpatient treatment is required or if the patient can safely be treated at home.

SummaryJiménez D, et al.  Simplification of the pulmonary embolism severity index for prognostication in patients with acute symptomatic pulmonary embolism.  Arch Intern Med. 2010 Aug 9;170(15):1383-9. doi: 10.1001/archinternmed.2010.199.
The simplified PESI predicts 30-day mortality risk with accuracy similar to PESI.  Investigators condensed the 11 PESI criteria to 7, each worth 1 point:  aged > 80 years old, history of cancer, history of chronic lung disease or heart failure (2 factors combined), pulse ≥ 110 beats per minute, systolic blood pressure (SBP) < 100 mm Hg, arterial oxygen saturation < 90%.    A score = 0 is low-risk and a score ≥ 1 is high-risk.

For information on the original PESI:
Aujesky D, et al.  Derivation and validation of a prognostic model for pulmonary embolism.  Am J Respir Crit Care Med. 2005 Oct 15;172(8):1041-6. Epub 2005 Jul 14.
Investigators applied point values for 11 factors to categorize 30-day mortality rate for 10,354 patients in the derivation cohort.   Rates of 30-day mortality based on total score, ranged from Class I (very low risk) to Class V (very high risk).  View the 11 factors with the DynaMed PESI Calculator (From the Calculators menu, select Clinical Criteria.)

For complete summary and to see results from validation cohort studies, see Clinical Prediction of Pulmonary Embolism in DynaMed.

jkn 2/16