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| CASE REPORT |
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| Year : 2019 | Volume
: 2
| Issue : 2 | Page : 71-73 |
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Primary Pleural Primitive Neuroectodermal Tumor: Incidental Diagnosis of a Rare Pathology
Prriya B Eshpuniyani1, Ramakant Deshpande1, Kumar Doshi2, Sameer Pathan3, Sagar Sharma1
1 Department of Surgical Oncology, Asian Cancer Institute, Mumbai, Maharashtra, India
2 Department of Pulmonary Medicine, Asian Cancer Institute, Mumbai, Maharashtra, India
3 Department of Histopathology, Asian Cancer Institute, Mumbai, Maharashtra, India
| Date of Web Publication | 30-Dec-2019 |
Correspondence Address:
Dr. Prriya B Eshpuniyani
Department of Surgical Oncology, Asian Cancer Institute, Off Easter Express Highway, Behind Everard Nagar, Somaiya Ayurvihar, Sion (East), Mumbai 400022, Maharashtra.
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jco.jco_7_19
Primitive neuroectodermal tumors (PNETs), a part of Ewing’s sarcoma family of tumors (ESFT), are small round cell tumors, arising from embryonic neural crest cells. They usually present with chest pain , and dyspnea. There is tenderness of chest wall. Primary pleural PNETS are a rare entity. We present a rare case of primary PNET which was incidentally diagnosed in a young patient with blunt chest trauma. Keywords: Incidental diagnosis, primary pleural primitive neuroectodermal tumor, rare entity
How to cite this article:
Eshpuniyani PB, Deshpande R, Doshi K, Pathan S, Sharma S. Primary Pleural Primitive Neuroectodermal Tumor: Incidental Diagnosis of a Rare Pathology. J Curr Oncol 2019;2:71-3 |
| Introduction | |  |
Primitive neuroectodermal tumors (PNETs), a part of Ewing's sarcoma family of tumors (ESFT), are small round cell tumors,[1],[2] arising from embryonic neural crest cells.[1],[3] PNET of the chest wall may affect the ribs, sternum, scapula, clavicle, and soft tissues of the chest wall and pleura.[3] Primary pleural PNET is a very rare entity.[4]
We report a rare case of primary pleural PNET discovered incidentally in a 21-year-old gentleman who had presented with blunt chest trauma. Therapeutic video-assisted thoracoscopy (VATS) evaluation proved it to be pleural PNET. Detailed investigations revealed no other focus of the pathology other than pleura.
| Case Report | | |
A 21-year-old gentleman was admitted for traumatic hemothorax with normal vitals. X-ray of chest showed haziness of left chest. VATS evaluation showed only extensive blood clots and soft tissue debris. Final histopathology (HPE) revealed a cellular undifferentiated tumor that composed of sheets of small round uniform cells divided into vague lobules by thin fibrovascular septa. The cells possessed round to oval nuclei with occasional prominent nucleoli and a granular chromatin pattern. They possessed a clear to acidophilic cytoplasm. Mitotic activity was low. No atypical forms, necrosis, or heterologous elements were noted. Diagnosis of PNET was confirmed by immunohistochemistry (IHC): CD 99, FL-1, and BCL-2 positive. Positron emission tomography–computed tomography (PET–CT) scan showed mild left pleural effusion with lobulated hypo-enhancing pleural based soft tissue deposit in left lung base with no uptake elsewhere [Figure 1]. He underwent neoadjuvant chemotherapy followed by total pleurectomy with an uneventful postoperative recovery. HPE and IHC report confirmed deposits of PNET over left pleura [Figure 2]. He received adjuvant chemoradiotherapy as per Ewing's protocol. PET–CT at the end of treatment showed only minimal non-avid pleural effusion with no other evidence of metastases in the study [Figure 3]. | Figure 1: Preoperative PET–CT: Lobulated hypo-enhancing pleural based soft tissue deposit in left lung base with no uptake elsewhere
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,  | Figure 2: HPE: IHC of tumor cells exhibiting CD 99 positivity, high power view
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,
No evidence of disease was observed after 18 months of primary surgery followed by chemoradiotherapy.
| Discussion | | |
PNETs are a part of ESFT. They are small round cell tumors,[1],[2] arising from embryonic neural crest cells.[1],[3] PNET of the chest wall usually involves chest wall, ribs, and thoracic cavity,[4] and is extremely malignant in nature.[2],[4] Patients may have pulmonary involvement along with a chest wall mass[1] and pleural involvement.[3] Pleura is rarely involved as the primary site.[4]
Frequently, PNET affects children and adolescents, with incidence peak between 15 and 25 years.[3],[5]
Common presentation is chest pain (that is tender on palpation) and dyspnea.[3],[4]
Our patient is a 21-year-old gentleman who presented with blunt chest trauma and who was incidentally discovered to have primary pleural PNET during evaluation.
Radiologically, these tumors are difficult to differentiate from other thoracic neoplasms.[4] Most common CT finding is a mass with heterogeneous contrast uptake.[3] In primary pleural PNET, PET–CT shows no FDG uptake in other areas except the pleura.[4] Distant metastases in these patients are usually seen in lungs, bones, bone marrow, liver, and brain.[5]
In our patient, whole body PET scan showed mild left-sided pleural effusion with lobulated hypo-enhancing pleural based soft tissue deposit in left lung base with no uptake elsewhere.
Therapy involves combinations of neoadjuvant chemotherapy, radical surgical resection, and adjuvant chemoradiation.[2],[3],[4] Surgery should be performed after chemotherapy, even if there is a complete response, because scattered malignant cells may still be found in the scar tissue.[2] High risk of both local recurrence and distant metastases makes postoperative chemoradiotherapy mandatory.[1]
Our patient underwent total pleurectomy after neoadjuvant chemotherapy. It was then followed up with adjuvant chemoradiotherapy.
The histopathological differential diagnosis of PNET of the lung includes small cell carcinoma and other small round cell tumors.[4] CD 99 is generally expressed in these tumors in IHC though it is not specific for PNET or Ewing's sarcoma.[2]
In our patient, HPE showed deposits of PNET over the left pleura. Diagnosis was confirmed by IHC, which showed CD 99, FL-1, and BCL-2 to be positive.
PNET prognosis is related with adjacent structures invasion,[5] and it is a highly malignant tumor with a very poor prognosis.[2]
PET–CT performed at the end of treatment after completion of adjuvant chemoradiotherapy shows no evidence of disease.
A rare case of incidentally detected primary pleural PNET, during evaluation of a traumatic hemothorax, is herein reported.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Rakesh R. Peripheral primitive neuroectodermal tumor of chest wall. Indian J Appl Res2015;5:34-36.  |
| 2. | Kubra Erol K, Ahmet B, Fatih S, Nurcan U, Mahmut Y. Thoracic primitive neuroectodermal tumour: An unusual case and literature review. Case Reports Pulmonol 2013;2013:326871. |
| 3. | Bruno H, José Carlos F, Edson M. Uncommon intrafissural mass: Pleural primitive neuroectodermal tumour. Arch Bronconeumol 2015;51:46-54. |
| 4. | Johari M, Prajapat DK, Khanna A, Talwar D. Primary pleural primitive neuroendocrine tumor: A rare entity. J Assoc Chest Physicians 2016;4:81-3. [Full text] |
| 5. | Gilmar Moreira SJ, Gláucia M, Ribeiro Z, Jorge Luiz B, Edson M. Peripheral primitive neuroectodermal tumour of chest wall in young adult. Radiol Bras 2015;48:59-64. |
[Figure 1], [Figure 2], [Figure 3]
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