Carcinoid tumor in a 10-year-old boy challenges in diagnosis and management: A rare case report

Abstract

Introduction

Lung cancer is a major cause of cancer-related deaths, with 2 million new cases annually. Bronchopulmonary neuroendocrine tumors (BP-NETs) comprise about 20 % of lung cancers, including typical carcinoid tumors (TC). While tobacco is a primary risk factor, non-tobacco factors also play a role. Typically, NETs affect individuals over 50, with typical carcinoids being rare in children. This report discusses a unique case of a typical carcinoid in a 10-year-old boy linked to environmental exposure.

Case presentation

A 10-year-old boy presented with cough, dyspnea, hemoptysis, and recurrent pneumonia. Imaging revealed lobar collapse and a large mass in the right lower lobe. Surgical excision confirmed a carcinoid tumor with no lymph node metastasis. Post-surgery, the patient showed complete symptom resolution during follow-up.

Discussion

Neuroendocrine tumors (NETs) are uncommon in children and can present with respiratory symptoms. This case highlights the importance of considering NETs in pediatric patients, particularly with environmental exposure. Timely diagnosis and surgical intervention led to a successful outcome, emphasizing the need for awareness of these tumors in younger populations.

Conclusion

This case report presents a rare typical carcinoid tumor in a 10-year-old boy, with successful surgical resection leading to complete symptom resolution. It highlights the need to recognize bronchopulmonary neuroendocrine tumors in children, particularly those with environmental exposure, and underscores the importance of ongoing follow-up for affected patients.

Highlights

• •A rare typical carcinoid tumor in a 10-year-old boy challenges the notion that these tumors affect only older patients.
• •The patient presented respiratory symptoms and imaging findings that initially suggested a different diagnosis.
• •Surgical resection resulted in complete symptom resolution, highlighting the need for timely intervention.
• •This case highlights the importance of awareness bronchopulmonary neuroendocrine tumors in children.

1. Introduction

Lung cancer is one of the main reasons of cancer _related deaths with approximately 2 million new cases and 1•76 million deaths per year [1]. Bronchopulmonary neuroendocrine tumors (BP-NETs) represent approximately 20 % of all lung cancers and include a wide range of tumors arising from neuroendocrine cells of the BP-epithelium., they can be divided into 4 subgroups: typical carcinoid tumor (TC), atypical carcinoid tumor (AC), large-cell neuroendocrine carcinoma (LCNEC), and small-cell lung carcinoma (SCLC) [2]. most of the studies focus on Tobacco as major risk factor for lung cancer. Nontobacco risk factors may include environmental and occupational exposures, chronic lung disease, lung infections, and lifestyle factors [3].therefor most studies show that NET usually tend to affect patients, who are older than 50 years and unlikely to occur in younger patient especially typical carcinoid, which represent approximately 2 % of lung NETs [4] We report here a very rare case of a typical carcinoid, that occurs in a child of only 10 years old due to environmental exposure, and we highlight the challenges encountered in managing this unique case as per the SCARE checklist [5].

2. Case presentation

A ten-year-old boy presented to the hospital with a history of cough, episodes of dyspnea, occasional hemoptysis, recurrent lobar pneumonia, paroxysmal tachycardia, and episodic facial flushing and erythema, with no family or medical history. His physical examination showed that his pulse was 92 beats per minute, respiratory rate 14 breaths per minute, po2 was 95 mmHg, GCS score was 15/15, and upon auscultation, there was a notable reduction in breath sounds at the right lung base. The chest radiography revealed signs of lobar collapse in the medial segment of the right lower lobe, with compensatory hyperinflation in the right lung, and fine inflammatory densities in the medial aspects of both lungs (Fig. 1).

Fig. 1.

An chest X-ray showed indications of lobar collapse in the medial segment of the right lower lobe, along with compensatory hyperinflation in the right lung, and subtle inflammatory densities in the medial areas of both lungs.

The chest and mediastinum were studied with computer-assisted CT sections, which showed: A consolidation collapse appearance in the right lower lobe and obstruction in the right lower bronchus with a small amount of right pleural effusion and mild condensation with bronchial impressions in the right middle lobe. No signs of a mass or abnormal pulmonary densities. Additionally, there is no enlargement of the mediastinal or hilar lymph nodes, nor is there any left pleural effusion (Fig. 2).

Fig. 2.

An CT scan of the chest and mediastinum revealed a consolidation collapse in the right lower lobe, along with an obstruction in the right lower bronchus. There was a small amount of right pleural effusion and mild condensation with bronchial impressions noted in the right middle lobe. Importantly, there were no signs of a mass or abnormal pulmonary densities. Furthermore, there was no enlargement of the mediastinal or umbilical lymph nodes, and no left pleural effusion was observed.

The bronchioalveolar washing results manifested benign cytology:

• •Moderate cellularity with a hemorrhagic and inflammatory background.
• •Predominance of suppurative inflammation rich in neutrophils and histiocytes.
• •Epithelial cells predominate with metaplastic squamous cells without evident atypia.
• •Associated with a few bronchial and rare reactive pneumocytes.

The Computed Tomography (CT) with virtual bronchoscopy showed a large mass in the alveolar tissue measuring 9 cm, containing numerous punctate calcifications, involving the medial aspect of the right lower lobe and extends inferiorly to the right lung hilum, causing an almost complete obstruction of the lower branch of the right main bronchus. This is associated with elevation of the right diaphragm and a slight right pleural effusion (Fig. 3).

Fig. 3.

An Computed Tomography (CT) scan with virtual bronchoscopy identified a large mass in the alveolar tissue, measuring 9 cm. This mass, characterized by numerous punctate calcifications, is located in the medial aspect of the right lower lobe and extends inferiorly to the right lung hilum. It is causing nearly complete obstruction of the lower branch of the right main bronchus. Additionally, there is elevation of the right diaphragm and a slight right pleural effusion noted.

After one year of follow-up, the CT scan showed a decrease in the severity of condensation and collapse in the lower lobe of the left lung, with limited alveolar densities remaining around the bronchi. A tissue mass containing punctate calcifications with a maximum diameter of 2.5 cm, extends from the lateral edge of the mid-lower branch of the right main bronchus into this branch, causing near obstruction of the airway, suggestive of a teratoma (Fig. 4).

Fig. 4.

An CT scan revealed a reduction in the severity of consolidation and atelectasis in the lower lobe of the left lung, with only minimal alveolar densities persisting around the bronchi. Additionally, there is a tissue mass measuring up to 2.5 cm, characterized by punctate calcifications, which extends from the lateral edge of the mid-lower branch of the right main bronchus into this bronchial branch. This mass is causing near obstruction of the airway and is suggestive of a teratoma.

The patient was referred for surgical excision of the lower and middle lobes of the right lung after a year of continuous follow-up. The right lower pulmonary lobe had maximum dimensions of 12 × 3 × 8 cm and showed a retracted area at the hilum of the lobe upon sectioning. Its tissue was dense and poorly aerated, with a maximum diameter of 3.5 cm, and there were signs of diffuse bronchitis within the parenchyma of the lobe. The right middle pulmonary lobe measured a maximum of 4.5 × 7 × 8.5 cm and displayed a pale yellow, dense pulmonary parenchyma upon sectioning, compared to normally aerated lung tissue.The diagnosis was carcinoid tumor with trabecular bone formation, severe micro-abscessing bronchopneumonia, and no lymph node metastasis. The surgical pathology report confirmed that the histological changes were well- differentiated neuroendocrine tumor (Typical carcinoid tumor).The patient visits the hospital every six months for follow-up, and he was in great condition, all symptoms had resolved after the surgery and follow-up.

3. Discussion

Neuroendocrine tumors (NET) arise from spread endocrine cells. They can be found primary in 65 % of cases along the gastrointestinal tract and pancreas, then secondary in 25 % of cases in bronchopulmonary (BP) tree [6]. Neuroendocrine tumors have a wide range of malignant potential, account for about 0.5 % malignancies tumors. So, they can be classified into typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma based on a group of features [7]. NET usually tend to affect patients, who are older than 50 years and unlikely to occur in younger patient especially typical carcinoid, which represent approximately 2 % of lung NETs [4] We report here a very rare case of a typical carcinoid, that occurs in a child of only 10 years old. The symptoms of NET in the chest can vary depending on the histological classification and the site of the tumor. The majority are central tumors who appear symptomatic as recurrent infections, chest pain, cough, wheeze, dyspnea, and pneumonia. While the rest are located peripherally and are usually asymptomatic and tend to discover by chance. Carcinoid syndrome or hormone secretion are hardly present in lung NET [8]. In our case, the patient suffered generally from cough, recurrent pneumonia and dyspnea, in addition to that he has a hemoptysis, flashing on the cheek and tachycardia. In order to diagnose this tumor: We use chest X-ray as a first-line imaging investigation in most patients with suspected bronchial NETs, which usually appears abnormal in more than 40 % of cases. Then we use more sensitive investigations such as CT or magnetic resonance imaging. Bronchoscopy provides a chance for biopsy [8] In our case we notice in chest X-ray image: densities consistent with inflammation in the base of both lungs, along with atelectasis of the lower lobe in the right lung. In the CT we see: A mass tissue shows punctate calcifications with a maximum diameter of 2.5 cm extending from the lateral border of the middle of the lower branch of the right main bronchus to the inside of this branch, causing a partial of the airway. While in bronchoscopy we observed at the entrance to the right bronchi a Granular tissue, Multiple biopsy was taken. The preferred treatment for pulmonary carcinoids (PCs) is surgical removal. The goal is to excise the tumor while preserving as much lung tissue as possible. The surgical method chosen depends on the tumor's size, location, and type of tissue involved [9]. In our patient the middle and lower lobes of the right lung were resected in a patient with obstructive syndrome.

4. Conclusion

This case report highlights a rare instance of a typical carcinoid tumor in a 10-year-old boy, emphasizing the occurrence of bronchopulmonary neuroendocrine tumors in pediatric patients. The patient's respiratory symptoms and imaging findings led to a successful diagnosis and surgical resection, resulting in complete resolution of symptoms. This case underscores the need for awareness of BP-NETs in children, particularly with a history of environmental exposure, and the importance of ongoing follow-up for affected patients.

CRediT authorship contribution statement

The work's conception and design: all authors. Paper writing, and article revision: all authors. Final revision and approval: all authors.

Informed consent

Unnecessary, information taken from the patient's file.

Ethical approval

Single case reports are exempt from ethical approval in our institution(University of Aleppo), as the paper does not contain any information that identifies the patient. Moreover, Informed consent was obtained from the patient.

Guarantor

Tameem Azzawi.

Research registration number

Our research study does not involve human subjects.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Consent for publication

All authors provide consent for publication.

Funding

There are no funding sources.

Declaration of competing interest

The authors declare that they have no competing interests.