[ CHEST Pearls ]

A Young Child With Recurrent Pneumonia

and Hemoptysis During the COVID-19
Pandemic
Zirun Zhao, MD; Rachel Choe Kim; Felix Tavernier, MD; Rachana Choksi, MD; Trevor Van Brunt; James Earl Davis, MD;
Katharine Kevill, MD; and Helen Hsieh, MD, PhD

CASE PRESENTATION: In July 2020, a previously healthy 6-year-old boy was evaluated in a
pulmonary clinic in New York after two episodes of pneumonia in the previous 3 months.
For each episode, the patient presented with cough, fever, and hemoptysis, all of which
resolved with antibiotic therapy and supportive care. The patient never experienced dyspnea
during these episodes of pneumonia. He was asymptomatic at the current visit. The patient
had no history of travel, sick contacts, asthma, or bleeding disorders.
CHEST 2022; 162(2):e77-e80

Physical Examination polymerase chain reaction was negative. Point-of-care

On examination, the patient was afebrile, normotensive, hemoglobin increased to 11.6 g/dL 1 month later. Chest
and had a respiratory rate of 16 breaths per minute and radiographs demonstrated a right lower lobe opacity at
an oxygen saturation >99% on room air. Lungs were the initial episode and again at the second episode of
clear to auscultation bilaterally. Chest wall excursion was pneumonia 2 months later.
equal bilaterally. He had no rash, petechiae, or joint
One week after the current clinic visit (July 2020), a
swelling. Results of the rest of the physical examination
repeat hemoglobin was 7.0 g/dL with a mean
were within normal limits.
corpuscular volume of 70.4 fL and red cell distribution
width of 21.1%. A repeat chest radiograph performed
Diagnostic Studies while the patient asymptomatic again demonstrated a
At the initial presentation of pneumonia (April 2020), persistent right lower lobe opacity (Fig 1).
laboratory results were significant for a hemoglobin of
9.6 g/dL, elevated WBC 15.9 k/mL, D-dimer 879 ng/mL, What Study Should Be Conducted Next?
and C-reactive protein 1.2 mg/dL. COVID-19 CT chest with and without IV contrast

AFFILIATIONS: From the Renaissance School of Medicine (Z. Zhao, CORRESPONDENCE TO: Helen Hsieh, MD, PhD; email: helen.hsieh@
R. C. Kim. T. Van Brunt), the Department of Pathology (F. Tavernier, stonybrookmedicine.edu
R. Choksi, J. E. Davis), the Department of Pediatrics, Division of Copyright Ó 2022 American College of Chest Physicians. Published by
Pulmonology (K. Kevill), and the Department of Surgery, Division of Elsevier Inc. All rights reserved.
Pediatric Surgery (H. Hsieh), Stony Brook University, Stony Brook, DOI: https://doi.org/10.1016/j.chest.2022.03.053
NY.

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Figure 1 – Chest radiography demonstrating right lower lobe opacity in (A) Initial chest radiograph and (B) chest radiograph at 2 months.

What is the diagnosis?

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 High-grade MEC demonstrates nuclear pleomorphism,
Diagnosis: Endobronchial mucoepidermoid abnormal mitotic activity, and necrosis and is associated
carcinoma (MEC) with increased metastasis and mortality. Low-grade
MEC has a complete absence of these histologic
Discussion characteristics and has a good clinical outcome.
The differential diagnosis for recurrent pneumonia in Diagnosis of MEC and other endobronchial lesions
children is wide; causes can include immunodeficiency, occurs, on average, 11 months after symptom onset.
impaired mucociliary clearance, mechanical airway Diagnosis is based on clinical findings of bronchial
obstruction, systemic and immune-mediated disease, obstruction, chest radiographs, and chest CT. Large
congenital pulmonary airway malformation/ endobronchial lesions cause bronchial obstruction and
sequestration, and nonpulmonary causes. Involvement frequently present as recurrent obstructive pneumonia
of tumors or surgical solutions are rare. Recurrent distal to the lesion. Other clinical manifestations of large
pneumonia associated with a persistent lesion on airway involvement include hemoptysis, cough, wheezing,
imaging warrants cross-sectional imaging to evaluate for bronchitis, chest pain, and clubbed fingers. CT is the most
anatomical abnormalities. sensitive and specific examination to detect parenchymal
lung disease; therefore, it is the test of choice if a mass is
Endobronchial tumors, as described in this case, are rare
suspected. PET-CT also can be useful to determine
in the pediatric population. Such tumors have mostly
metastasis, and the use of Gallium-68 may help
been described in case reports or in small case series.
distinguish the lesion from carcinoid tumor. Serum
The largest case series, comprising 14 total patients,
markers such as chromogranin A and 5-
included five carcinoid tumors, three mucoepidermoid
hydroxyindoleacetic acid also may help identify carcinoid
carcinomas, and one adenoid cystic carcinoma.
tumors. Flexible fiber-optic bronchoscopy is useful for
MEC accounts for only 0.1% of primary lung tumors in visualization of the mass; however, MEC lesions are friable
patients across the age spectrum. It is the most common and may result in bleeding after biopsy or manipulation.
malignant tumor of the salivary gland in adults but also
Surgical resection is the treatment of choice for
can be found in the thyroid, lacrimal gland, and the
bronchial MEC, with a primary goal of negative surgical
lung. This tumor is most often seen in young adults;
margins. In children and adolescents, 5-year survival is
however, it has been reported in patients ranging from 3
96%. Local recurrence is extremely rare and limited to
to 72 years of age. In children, bronchial MEC occurs in
high-grade disease. In one case series, five deaths out of
10% of malignant lung tumors. Only 145 pediatric cases
120 cases (4%) resulted from metastatic disease
of MEC are reported in literature.
secondary to high-grade tumor. Adjuvant chemotherapy
Definitive diagnosis of MEC is by or radiation therapy are usually not indicated when
immunohistochemistry and histology. Although MEC is complete resection was achieved because of the benign
normally a salivary gland tumor, it can originate from clinical course of MEC.
large airways, such as the trachea and primary/
secondary bronchi. MEC is a slow-growing, firm,
unencapsulated tumor with low potential for Clinical Course
malignancy. Histologically MEC contains three cell CT chest revealed a 3.1  2.3  2.1-cm mass in the right
types: squamous, mucus secreting, and intermediate lower lobe bronchus with postobstructive mucoceles and
cells. Immunohistochemistry is positive for CK7, bronchiectasis (e-Fig 1). A subsequent PET-CT scan
MUc5Ac, p40, and p63 and negative for thyroid with Gallium-68 demonstrated moderate tracer uptake
transcription factor 1, calponin, human epidermal and no sign of metastatic disease. Chromogranin A was
growth factor receptor 2, and anaplastic lymphoma normal. Surgical resection was delayed by a third
kinase. These markers distinguish bronchial MEC from episode of hemoptysis, fever, and dyspnea, which
other primary lung tumors. Mastermind like improved with IV antibiotics. Preoperative flexible
transcriptional coactivator 2 (MAML2) is the most bronchoscopy demonstrated an endobronchial mass
frequently identified gene rearrangement associated with distal to the right middle lobe bronchus. The patient
MEC and occurs in 77% of the cases, whereas epidermal underwent an uncomplicated right middle and lower
growth factor receptor mutations also have been lobectomy 5 months after initial presentation, with
reported. Prognosis is determined by histological grade. complete resolution of symptoms.

chestjournal.org e79
Histopathology revealed a low-grade, mucus-secreting Other contributions: CHEST worked with the authors to ensure that
the Journal policies on patient consent to report information were met.
tumor (e-Fig 2) positive for CK7, and negative for
Additional information: The e-Figures are available online under
thyroid transcription factor 1, CK20, and p63. A positive “Supplementary Data.”
MAML2 (11q21) rearrangement confirmed the
diagnosis of MEC. After an uneventful recovery from Suggested Readings
surgery, the patient was asymptomatic at 1-year follow- Al-Qahtani AR, Di Lorenzo M, Yazbeck S. Endobronchial tumors in
children: institutional experience and literature review. J Pediatr Surg.
up. All laboratory values have normalized. Repeat CT 2003;38(5):733-736.
does not demonstrate recurrence of disease. Achcar Rde O, Nikiforova MN, Dacic S, et al. Mammalian mastermind
like 2 11q21 gene rearrangement in bronchopulmonary
mucoepidermoid carcinoma. Hum Pathol. 2009;40(6):854-860.
Clinical Pearls
Roby BB, Drehner D, Sidman JD. Pediatric tracheal and endobronchial
1. In the setting of recurrent childhood pneumonia with tumors: an institutional experience. Arch Otolaryngol Head Neck Surg.
uncommon clinical findings, including hemoptysis, 2011;137(9):925-929.
Stillwell PC. Recurrent pneumonia. In: Schechter MS, ed. Pediatric
profound anemia, lack of dyspnea, and a persistent Pulmonology: American Academy of Pediatrics; 2011:451-458.
opacity/mass on imaging, rare diagnoses such as an Alsidawi S, Morris JC, Wikenheiser-Brokamp KA, et al. Mucoepidermoid
endobronchial mass should be included in the differ- carcinoma of the lung: a case report and literature review. Case Rep Oncol
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Qian X, Sun Z, Pan W, et al. Childhood bronchial mucoepidermoid
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1409-1412.
flexible bronchoscopy. Tumor markers and the
Eyssartier E, Ang P, Bonnemaison E, et al. Characteristics of
MAML2 gene rearrangements can distinguish MEC endobronchial primitive tumors in children. Pediatr Pulmonol.
from other primary lung tumors. 2014;49(6):E121-E125.
Huo Z, Wu H, Li J, et al. Primary pulmonary mucoepidermoid
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tive treatment for bronchial MEC, with excellent long- PLoS One. 2015;10(11):e0143169.

term prognosis. Jaramillo S, Rojas Y, Slater BJ, et al. Childhood and adolescent
tracheobronchial mucoepidermoid carcinoma (MEC): a case-series and
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Acknowledgments Jichlinski A, Kilaikode S, Koumbourlis AC. Case 1: recurrent pneumonia
Financial/nonfinancial disclosure: None declared. in a 15-year-old girl. Pediatr Rev. 2018;39(9):464.

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