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\begin{document}

\title{Pulmonary Inflammatory Myofibroblastic Tumor in a Male Child; a Case
Report}



\author[1]{Seyed Javad  Seyedi}%
\author[1]{Amin Saeidinia}%
\author[1]{Parisa Dehghanian}%
\affil[1]{Mashhad University of Medical Sciences}%


\vspace{-1em}



  
  \date{June 17, 2024}


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\selectlanguage{english}
\begin{abstract}
Pulmonary inflammatory myo-fibroblastic tumor (IMT) is a rare condition
in normal population and specifically in pediatric population. We
reported a 9-year-old male child presented by cough and intermittent
fever and weight-loss that was mostly suggestive for infectious process.
We reviewed the consideration of diagnosis and treatment and other
previous cases.%
\end{abstract}%



\sloppy


\textbf{Pulmonary Inflammatory Myofibroblastic Tumor in a Male Child; a
Case Report}

\textbf{Seyed-Javad Seyedi\textsuperscript{1}, Amin
Saeidinia\textsuperscript{1,2*}, Parisa Dehghanian\textsuperscript{3}}

\emph{1. Pediatric Department, Faculty of Medicine, Mashhad University
of Medical Sciences, Mashhad, Iran}

\emph{2. Pharmaceutical Research center, Mashhad University of Medical
Sciences, Mashhad, Iran}

\emph{3. Pathology department, Akbar Hospital, Mashhad University of
Medical Sciences, Mashhad, Iran}

\textbf{Abstract}

Pulmonary inflammatory myo-fibroblastic tumor (IMT) is a rare condition
in normal population and specifically in pediatric population. We
reported a 9-year-old male child presented by cough and intermittent
fever and weight-loss that was mostly suggestive for infectious process.
We reviewed the consideration of diagnosis and treatment and other
previous cases.

\textbf{Keywords:} inflammatory myo-fibroblastic tumor, pulmonary tumor,
pediatric population

*Corresponding author: Fifth floor, Akbar hospital, Kaveh Boulevard,
Javan Square, Mashhad, Iran; Email: saeidiniaa971@mums.ac.ir, Tel:
+989119451607

\textbf{Background}

Occurrence of primary pulmonary neoplasms in pediatric patients are
uncommon. Inflammatory myo-fibroblastic tumor (IMT) is a rare neoplasm,
which has a moderate risk of malignancy appearing mainly in children and
young adults (1, 2). Its nature is in majority of cases benign and
rarely can metastases however could commonly relapse (3). This tumor is
consist of different inflammatory cells, which its diversity is from
primarily myo-fibroblastic cells to plasma cells in pathologic exam (4).
IMT is defined as ``a lesion composed of a proliferation of
myo-fibroblastic spindle and stellate cells with abundant eosinophilic
cytoplasm mixed with infiltrative plasma, inflammatory cells,
lymphocytes and eosinophils'' in world health organization (WHO) (5).
IMT is known by numerous terms, including plasma cell granulomas,
inflammatory pseudo-tumor, fibrous histiocytoma and pseudo-lymphoma (6),
and may occur in a wide range of anatomical locations such as the lungs,
omentum, bladder, spleen, breast, pancreas, liver, colon, spermatic
cord, prostate, peripheral nerves, soft tissue, and orbit. About
one-third of these tumors are found in the respiratory tract (6). We
here report a case of IMT of the lung.

\textbf{Case presentation}

A 9-year-old male child with chronic productive cough and occasional
fever since six months ago referred to our clinic. He had severe weight
loss in this period. Two months before admission, he had massive
hemoptysis and clubbing. There was no shortness of breath or Respiratory
distress. He had a history of tonsillectomy 2 years ago and no exposure
to tuberculosis case. Theophylline, Guaifenesin and Cefixime have been
administered for him. There no history of familial pulmonary disorder.
At admission, his vital signs were stable. In the examination, there was
decreased pulmonary sound in right lung. There was clubbing in upper and
lower limbs fingers. His chest x-ray is shown in figure1. In his
pre-admission computer tomography (CT) scan, a mass-like foci in the
right lung was reported.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure1- Chest X Ray}

He was admitted in our center for more evaluation. After admission
antibiotic therapy and supportive care was performed. He underwent
bronchoscopy. In fibro-optic bronchoscopy, upper division of right
bronchus was completely obstructed. After washing, various cystic
membranes was seen in the airway. The mucosa was erythematous and
inflamed with dense secretions that was suctioned (Figure2).\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Figure2- Bronchoscopy; the upper division of right bronchus that
is obstructed by a mass.}

There was no pathologic issue in his chest and abdominal sonography. His
first laboratory data showed a leukocytosis (WBC 22,300 with PMN
dominant pattern (85\%)). ESR and CRP was in nearly normal range. Anti
Echinococus antibody was negative. Other laboratory data was in normal
range. After surgical consult, he was candidate for surgery and
underwent right upper lobectomy and wedge resection. After thoracotomy,
mass-like lesion with granulomatous tissue was resected. There was a
fistula that was closed by resection. Chest tube size 28 was inserted
and the sample was sent for pathologic examination.

Pathologist assessed wedge biopsy of lung, with brown outer surface,
measuring 2.5\selectlanguage{ngerman}×2 ×1 cm, and a central creamy-yellow nodular lesion,
measuring 1 cm in diameter. Sections of the lung tissue with pleural
fibrous thickening, revealing prominent interstitial nodular lymphoid
proliferations surrounding large epithelioid cells (resembling
granulomas), few of them with large nucleolated cells without necrosis.
A diffused intra-alveolar infiltration of foamy histiocytes were noted.
In addition, a central focus of pulmonary necrosis with mixed acute
inflammatory cell infiltration (abscess formation) was present. Foci of
hemorrhage was also repoted. In Zhiel-Neelson staining acid-fast bacilli
were not seen. PPD test was negative. Because of nodular lymphoid
proliferation of pulmonary interstitium with one necrotic area and
abscess formation, it was suggestive for interstitial lymphocytic
pneumonia or granulomatous disease and we ruled out it by checking CD20,
CD3, CD15 and CD30 that shown no definite evidence of Hodgkin lymphoma.
Inflammatory myo-fibroblastic tumor (IMT) was diagnosed by its pattern
(Figure3). Patient discharged after the surgery and is following up to
now for probable relapse.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image5/image5}

\end{center}
\end{figure}

\textbf{Figure3- Histopathological H\&E staining showing inflammatory
cells}

\textbf{Discussion}

IMT is one of the rare low-to-intermediate grade sarcomas. Initially it
was thought to be an inflammatory response to various stimuli, but
recent studies have proved IMT to be neoplastic and can recur locally
and metastasize (7). It has also been suggested that trauma, surgery,
autoimmune etiologies, inflammation, and infections such as Epstein-Barr
virus or human herpes virus could result in the development of IMT (8).

IMT was first described in the lungs but later was also found in other
sites such as the orbit, spleen, genitourinary tract, mesentery,
cardioesophageal junction, breast, central nervous system, and larynx.
The larynx has been a very rare site for involvement in IMT (9).
Children with IMT may exhibit symptoms of chronic inflammation as a
low-grade fever, weight loss, anemia, thrombocytosis, polyclonal
hyper-gammaglobulinemia, and elevated sedimentation rate. Several cases
are asymptomatic and are detected only incidentally on imaging studies.
Among patients with end-obronchial lesions, symptoms of bronchial
irritation such as cough and hemoptysis may be accompanied by chest pain
(10).

In our case, chest x-ray, history of weight loss and occurrence of
intermittent fever and hemoptysis, beside endemic status, made us
suspicious to infectious process like tuberculosis or echinococcosis.
Because of the negative staining of BAL sample and laboratory data, we
thought about the non-infectious process like malignancies because of
weight loss. Despite of doing bronchoscopy and HRCT, the diagnosis was
last after histopathological assessment. According to previous studies,
there are only 26 published cases of pediatric pulmonary IMT (the age
between 3 to 13 years), even though the real incidence is presumed to be
higher (3, 11). Peripheral lung lesions appear to be more frequent than
central and endobronchial tumors that may be present about in 10\% of
the cases resulting in bronchial obstruction and atelectasis (12).

IMT can be sometimes diagnosed as incidental finding on a routine CXR
(13). In all previous cases, at the time of presentation, patients had
fever, respiratory distress, arthralgia, clubbing, night sweat,
vomiting, and hemoptysis and at the onset, fever and cough were the
commonest symptoms (3). There has been an ongoing controversy whether an
IMT is a reactive lesion or a true neoplasm (14). Although its incidence
is fairly scarce, the existing literature clearly defines its relative
similarities in terms of clinico-pathological and radiological findings
and almost uniformly favors surgical resection as a mainstay for the
most efficient management strategy. The recurrence rate remains low and
a 10-year survival rate is around 80\% (15).

Treatment is primarily a complete but conservative surgical excision.
This approach is necessary to prevent recurrence (16, 17). An
appropriate histologic assessment should be obtained before the surgery
(needle biopsy by bronchoscopy), in order to avoid an unnecessarily
procedure (17).

\textbf{Conclusion}

Pulmonary IMT is a rare disorder with significant complications among
the children population. It may be non-specific in presentation in this
population and patients undergo different antibiotic treatment before
definite diagnosis. While radiological techniques can help in diagnosis,
confirmation of the diagnosis should be performed by histopathological
assessment. The choice of treatment is based on complete surgical
resection.

\textbf{Conflict of interest}

There is no conflict of interest.

We declare that none of the authors listed on the manuscript are
employed by a government agency that has a primary function other than
research and/or education. None of the authors is submitting this
manuscript as an official representative or on behalf of the government.

\textbf{Acknowledgement}

Nothing.

\textbf{Key clinical message}

Cough is a serious sign in children that should be regarded by
physicians. Pulmonary IMT may be non-specific in presentation in this
population. Despite of its rare prevalence, pulmonary mass like IMT
should be considered in the list of differential diagnosis of cough.

\textbf{Author contribution}

Dr. Seyed-Javad Seyedi, Dr. Amin Saeidinia and Dr. Parisa Dehghanian
were responsible for the case treatment, development of methodology,
writing of the manuscript and confirming final version.

\textbf{Data availability statement}

The data that support the findings of this study are available from the
corresponding author upon reasonable request.

\textbf{Patient consent}

The authors have confirmed that patients' consent have been signed and
collected in accordance with the journal's patient consent policy.

\textbf{References}

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2. Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary
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