August 7 2013 Long Case HPI 50 yo woman from the Dominican republic presenting to pulmonary clinic for cough 45 years Symptoms may have started after a cold Worse in the summer no temporal relation to nightday ID: 776588
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Slide1
Marc FreimanWednesday Pulmonary ConferenceAugust 7, 2013
Long Case
Slide2HPI
50
yo
woman from the Dominican republic presenting to pulmonary clinic for cough 4-5 years
Symptoms may have started after a cold
? Worse in the summer, no temporal relation to night/day
Dry, non-productive
DOE 2-3 city blocks, 2 flights of stairs
ROS - Denies HA, sinus congestion, heartburn, reflux. Denies chest pain, palpitations,
orthopnea
, PND or edema
Slide3PMHx/Soc Hx
VitiligoDenies childhood asthma
From DR 3 years ago
Worked in paper shredding factory for 1 yr
Currently works in retail
Never smoker
No
EtOH
,
illicits
Slide4Physical exam
Afeb
P 95 130/84 96% RA; BMI 30 (150lbs, 5’)
General: comfortable
Clear, no wheeze. ? Crackles at bases bilaterally
Neck: supple, no masses, neck nodes not
palpable
CV: RRR No
m/r/g
No cervical LAD, neck supple
No
desaturation
on exertion
Slide5CXR
Slide6Slide7Symptomatic treatment
B
enadryl
Chlorpheniramine
Slide8Return visit
Benadryl lets her sleep through the night
Still with continued cough
She climbed
3 flights of stairs
and became SOB but did not
desaturate
- minimal
sats
96% HR 120
Slide9PFT
Slide10PFT
Slide11CT Scan
Slide12Slide13Slide14Slide15Slide16Slide17Slide18CT read
LUNGS
: There are
multiple nodules
in both lungs measuring
up to 5 mm
.
Mosaic
attenuation is seen in both lungs most prominent in the lower lobes suggestive of small airways or small vessel disease.
Slide19Labs
CBC,
Chem
7
wnl
ANA, RF negative
TTE unremarkable
Slide20Chronic cough
Slide21Slide22Just kidding…
Slide23VATS biopsy
Had
bronchoscopy
w
BAL
VATS biopsy for right lung with RML and RLL biopsy
Nodule palpated in RML
Slide24Bronchoscopy and VATS results
Middle lobe
lavage
cytology negative
Aerobic, anaerobic, fungal and AFB cultures negative
RIGHT
LOWER LOBE BIOPSY:
LUNG PARENCHYMA WITH CONGESTION, HEMORRHAGE AND HEMOSIDERIN LADEN MACROPHAGES.
NO TUMOR IDENTIFIED.
Slide25Slide26Slide27synaptophysin
Slide28chromogranin
Slide29RML biopsy
IMMUNOHISTOCHEMICAL STUDIES PERFORMED ON PARAFFIN EMBEDDED TISSUE (BLOCK A2) SHOWS POSITIVE STAINING FOR
CHROMOGRANIN, SYNAPTOPHYSINMULTIPLE FOCI OF NEUROENDOCRINE TUMOR, TUMORLETS/ SMALL CARCINOID TUMOR.
Slide30DIPNECH
Diffuse idiopathic pulmonary
neuroendocrine
cell hyperplasia
Slide31Overview of bronchopulmonary neuroendocrine tumors (BP-NET)
4 types
Typical
carcinoid
Atypical
Carcinoid
Large cell
neuroendocrine
carcinoma
Small cell
neuroendocrine
carcinoma
Slide32Diffuse Idiopathic Pulmonary Endocrine Cell Hyperplasia (DIPNECH)
Preneoplastic
Pulmonary
tumorlets
(<5mm)
Had been known to occur in:
ILD
B
ronchiolitis
obliterans
Patients living at
high altitudes
Slide33Purpose of the neuroendocrine cell in the lung?
UnknownArises from Kulchitsky cellThought to be involved as ‘airway sensors’Mediate airway tone, pulmonary circulation, and control of breathing.Act as both chemo and mechanoreceptorsAlso likely involved in development of the lung
Slide34Slide35AJRCCM - demographics
Women – 92% (23/25)
Mean diagnosis 58 years old
Range 36-76
67% non-smokers (16/24)
Slide36PFTs
Slide37CT findings
Pulmonary nodules (63%, 15 pts)
Ground glass (29%, 7 pts)
Bronchiectasis
(21%, 5 pts)
Mosaic attenuation (17%, 4 pts)
Slide38Clinical course – AJRCCM 2011
92% of patients had symptoms
Cough, dyspnea
, wheezing
Symptoms lasted between days to years – average 8.6
years
Widely variable course has been described
Not clear exactly why some people deteriorate – known to produce
bombesin
and
fibrinogenic
cytokines
41% (7) stable without clinical deterioration
Oral
predniosne
given to 2 of these patients
24% (4) clinically declined and didn’t improve
35% (6) declined but showed improvement clinically
Oral prednisone used in addition to bronchodilators in 4 of 6
No deaths
1 patient with asthma history who died of sepsis found to have DIPNECH on autopsy
Slide39Slide40Treatment
No formal evaluations of a treatment algorithm are available
Resection of dominant lesion
Oral/
inh
steroids
w
bronchodilators
Chemotherapy
Surgical lung resection
Presence of lymph nodes has not been associated
w
worse outcome
Lung transplantation (1pt, single lung, followed for 2 yrs)
Observation
?
Somatoastatin
analogues
Slide41Somatostatin-receptor scintigraphy (OctreoScan)
Tumors often express
somatostatin
Labeled
somatostatin
analog (
octreotide
)
Previously thought to be gold standard for diagnosis
Sn
approx 80-90%
Somatostatin
uptake may correspond to treatment response.
Somatostatin
targeted PET scan
Sn
as high as 100%, identified more lesions than SRS or CT
Slide42111In–DTPA–pentetreotide whole body scintigraphy (Octreoscan) showing an elective uptake of the radiolabeled octreotide in the liver, expression of distant localization of the neuroendocrine tumor of the lung (arrow).
Filosso
P L et al.
Eur J Cardiothorac Surg 2002;21:913-917
© 2002 Elsevier Science B.V.
Slide43Detail of the Octreoscan showing the liver metastase.
Filosso
P L et al.
Eur J Cardiothorac Surg 2002;21:913-917
© 2002 Elsevier Science B.V.
Slide44Patient’s octreotide scan
No uptake in the lungs
Increased uptake in the
cecum
Negative colonoscopy
Slide45Further investigation
Predisposing factors?
? Hormonal component
Tend to be middle-aged females
Unclear if race/ethnicity plays a role
Incidence?
Treatment algorithm?
Slide46References
Ann Oncol (2001) 12 (9): 1295-1300
.
Davies SJ,
Gosney
JR,
Hansell
DM, et al. Diffuse idiopathic
pul
-
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neuroendocrine
cell hyperplasia: an under-
recognised
spec-
trum
of disease. Thorax. 2007;62:249-252.
Cameron CM, Roberts F, Connell J,
Sproule
MW. Diffuse idiopathic pulmonary
neuroendocrine
cell hyperplasia: an unusual cause of cyclical ectopic
adrenocorticotrophic
syndrome. Br J
Radiol
. 2011;84:e14-e17.
25.
Fessler
MB, Cool CD, Miller YE, Schwarz MI, Brown KK.
Idio
-
pathic
diffuse hyperplasia of pulmonary
neuroendocrine
cells in a patient with
acromegaly
.
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K,
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Carcinoid
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Bronchopulmonary
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BI, Kidd M, Chan A,
Malfertheiner
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Signifi
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Surg
Pathol
. 1995;18:653-658.
Sheerin
N, Harrison NK, Sheppard MN,
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DM,
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Aguayo
SM, Miller YE, Waldron JA
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