Comprehensive treatment of diaphragm localized malignant mesothelioma and follow-up for five years—a case report and literature review.

doi:10.21203/rs.3.rs-1613396/v1

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Case Report

Comprehensive treatment of diaphragm localized malignant mesothelioma and follow-up for five years—a case report and literature review.

https://doi.org/10.21203/rs.3.rs-1613396/v1

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Background: Primary tumors of the diaphragm are rare,especially for the malignant mesothelioma.There is no case report of this kind of disease followed up for more than five years after surgical treatment in the world.

Case presentation:A patient presented with repeated chest tightness and shortness of breath for one year. A crescent shadow of the posterior costal diaphragm area with clear margin and obvious enhancement and higher signs of iodine photography was found on computed tomography.This patient was diagnosed by VATS(TV-assisted Thoracoscopic Surgery) and open thoracotomy surgery. After regular chemotherapy and 5 years of follow-up, the patient was in good condition and had no evidence of recurrence.

Conclusions: This case report can improve the awareness of the extremely rare diseases of malignant mesothelioma of the diaphragm. Early surgery combined with regular postoperative chemotherapy may give patients a good prognosis.

diaphragm

malignant mesothelioma

therapy

The primary tumor of diaphragm was first described in 1868 by Grancher, who found a fibroma of the diaphragm during autopsy[1].In the nearly 151 years from 1868 to 2019, there were nearly 200 total cases of diaphragmatic primary tumors reported worldwide, and only 8 of them were malignant mesothelioma[2-7].These patients were followed up for less than 2 years after surgery，We report a very rare case with malignant mesothelioma of the diaphragm after surgery and chemotherapy and five years of follow-up with no tumor recurrence.

A 51-year-old woman, without other medical problems, was admitted to our hospital with repeated chest tightness and tight breath for one year. She had been in previous good health, engaged in physical activity for a long time with no symptoms suggestive of cardiovascular disease, and had no history of hypertension, diabetes, or coronary artery disease, and without infectious diseases such as hepatitis, and exposure to special substances such as asbestos.The chest X-ray examination of the outpatient department did not find any special problems (Figure 1A). Physical examination and laboratory examinations such as tumor markers showed no significant abnormalities.Computed tomography (CT) of the thorax showed that there was an oval solid density shadow in the lower lobe of the left lung, with a maximum diameter of about 4.0cm, optical edges and unclear demarcation from the adjacent diaphragm and spleen (Figure 1B and Figure 1C).

After completing the preoperative preparation, the patient underwent thoracoscopic assisted mediastinal space occupying resection under general anesthesia.The tumor was isolated and located on the left diaphragm, with a size of about 4 *4 *2cm, oval, smooth as a whole and moderate hardness. There was a pedicle of about 1*1cm connected to the diaphragm. The blood supply of the mass is rich. After incision, the inner surface is smooth and flat, with lobulated changes and local "fish like" changes (Figure 2A).The tumor was completely removed and sent to freezing examination. The pathological diagnosis was malignant mesothelioma.The operation mode was changed and extended thoracotomy was performed .The abdominal cavity of diaphragm was smooth and flat, and there was no tumor invasion and metastasis around.The range of 8cm around the tumor pedicle was removed, and the diaphragm defect was repaired with 10 * 10cm diaphragm patch(Figure 2B).

The results of postoperative immunohistochemistry showed that: calretintin (+), CK (+), ck5/6 (+), vimentin (+), syn (-), CGA (-), desmin (-), SMA (-) CD34 (small blood vessels +) Diagnosis: malignant mesothelioma(Figure3).The patient was treated with cisplatin combined with docetaxel in the oncology department for three cycles. The patient was followed up to October 2021. There was no tumor recurrence and metastasis and survived healthily.

Malignant mesothelioma is a highly invasive disease, which originates from mesothelium, such as pleura, peritoneum, pericardium and so on. The most common is malignant pleural mesothelioma, accounting for about 80%，while malignant mesothelioma of the diaphragm is extremely rare[8]. The history of asbestos exposure is closely related to the occurrence of pleural mesotheliomas, but mesotheliomas in peritoneum, liver, diaphragm, and other organs have no obvious relationship with asbestos exposure history[9, 10].On the other hand, although the asbestos has been banned for decades in many countries, epidemiologists predict the malignant mesothelioma epidemic will last past 2040, raising many concerns in public health given its late diagnosis, dismal prognosis, and lack of current effective therapies[11, 12].Malignant mesothelioma can be divided into localized type and diffuse type according to the growth mode, and the prognosis of localized type is slightly better[13]. This patient is a rare case of localized malignant mesothelioma originating from the diaphragm.

These patients typically present with dyspnea in early stages.Then, as disease progresses, patients also complain of chest pain. The diagnosis is often made by thoracentesis as most patients have pleural fluid, which is positive for malignancy in 30% to 50% of patients.Computed tomography (CT) of the thorax and positron emission tomography scans is helpful in characterizing the tumor and its stage[3].The clinical prognosis for malignant mesothelioma is poor. Patients presenting with vague symptoms such as chest tightness and breathlessness should not be overlooked for lung pathology[14]. There was no obvious abnormality in the chest X-ray of this patient for one year. The space occupying of the left diaphragm was not found until the CT was completed before admission.Therefore, combined with the clinical manifestations of patients, early use of CT or MRI to improve chest tomography may be more beneficial to early diagnosis.

Currently, the incidence of primary tumor of the diaphragm is lower compared with that of secondary diaphragm tumor.Studies that explored primary diaphragm tumor previously are mainly case reports, and only a few systematic retrospective studies have been reported.Therefore the data is limited.There is no clear process or guideline for treatment of primary diaphragm tumor because of the low incidence, the various types of the disease, and the complex anatomic structures[15].Surgery is one part of multimodality treatment with resection of the diaphragmatic pleura or complete resection of the diaphragm to achieve macroscopic complete resection[16].When resecting diaphragmatic tumors the margins must be resected until negative even if this requires resection a moderate portion of the diaphragmatic surface.There are many ways to deal with the defective diaphragm in the world, which should be arranged according to the size and data of the defect[17, 18].The eight patients with malignant mesothelioma simply originating from the diaphragm mentioned above were all treated with surgery. Only one of the eight patients died of reexamination and metastasis during the two-year follow-up. One of them also had tumor recurrence during the postoperative follow-up, but the patient regained long-term survival after radiotherapy and chemotherapy.This patient was diagnosed as malignant tumor by thoracoscopy. Therefore, the diaphragm within about 8cm around the tumor pedicle was surgically removed, and the diaphragm patch was used to repair the defect. The patient received regular chemotherapy in the oncology department after surgery, and was followed up for five years, without any recurrence of the tumor, and without any discomfort.

For the symptoms of chest tightness and air tightness, excluding cardiopulmonary diseases, the primary tumor of diaphragm can not be ignored. At the same time, for the malignant mesothelioma of diaphragm, perhaps surgery combined with postoperative regular chemotherapy, patients may be able to obtain a better and longer-term survival.

Ethics approval and consent to participate

This is a case report that does not require ethics approval.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Availability of data and materials

Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.

Competing interests

The authors declare that they have no competing interests.

Funding

There was no funding for this case report.

Authors’ contributions

Qiu-lin, Chen completed data collection, follow-up,wrote the manuscript, obtained the written consent and gave final approval of the manuscript. All authors read and approved the final manuscript.

Acknowledgements

We thank all the staff of the department of Cardiothoracic Surgery, Guang yuan Central Hospital for their contributions to the acquisition of data and preparing the figures. None of the authors have any external funding source.

Author information

Affiliations

Department of Cardiothoracic Surgery, Chengdu Fifth People’s Hospital, No. 33, Mashi Street, Wenjiang District,Chengdu, Sichuan Province 611130, China.

Correspondence:[email protected].

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No competing interests reported.

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Comprehensive treatment of diaphragm localized malignant mesothelioma and follow-up for five years—a case report and literature review.

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