|Ahead of print publication
Gaurav Patel, Suyash Agrawal, Prakash Patil
Department of Surgical Oncology, Bombay Hospital and Medical Research Centre, Mumbai, Maharashtra, India
|Date of Submission||22-May-2019|
|Date of Acceptance||27-Nov-2019|
|Date of Web Publication||11-Jun-2020|
Department of Surgical Oncology, Bombay Hospital and Medical Research Centre, Mumbai - 400 020, Maharashtra
Source of Support: None, Conflict of Interest: None
We report the very rare case of recurrent unilateral pleural effusion in a 53-year-old male. Computed tomography (CT) scan and magnetic resonance imaging revealed a left-sided paravertebral mass at D3 level. Multiple biopsy and CT scan lead us to the diagnosis of “Angiomatous Malformation.” The lesion was excised surgically which on final histopathological report termed hemangioma.
Keywords: Effusion, hemangiomas, pleural, surgery, thoracic
| > Introduction|| |
Hemangiomas are neoplasms characterized by an increase in number of normal or abnormal vessels. It is a rare benign lesion and is thought to be associated with an imbalance of proangiogenic factors and angiogenesis inhibitors. It is usually a self-involuting kind of tumor. Hemangiomas constitute 7% of all benign tumors. Gindhart et al. reported that it is <0.5% of all mediastinal masses and up to 75% of these lesions manifest before the age of 35.
| > Case Report|| |
A 53-year-old male came to our clinic with left-sided intercostal drain (ICD) insitu, for 1 month, in view of repeated pleural effusion. On enquiry, the patient gives a history of breathlessness 2 months ago for which chest X-ray was done suggestive of massive pleural effusion on the left side. Thoracentesis was done for the same. Fluid according to notes was exudative and was send for routine/microscopy, cytology, and adenosine deaminase levels. These entire tests were within the normal limits. Later, a left-sided ICD was inserted for recurrent pleural effusions. Computer Tomography (CT) scan was done which showed a 7.1 x 7.1 x 6.9 cm well circumscribed extra pulmonary lesion in left paraspinal region at D3 level as shown in [Figure 1]. The lesion showed predominantly nonenhancing cystic component with few heterogeneously enhancing solid component extending just up to D3 neural foramina. Magnetic resonance imaging (MRI) confirmed the above lesion at D3 level. CT-guided biopsy was done which is termed as “Round-Cell Tumor.” Positron emission tomography (PET) scan was done for staging evaluation which said no fluorodeoxyglucose (FDG) uptake in the above said lesion. As the patient came to us with this biopsy and imaging findings, we were in doubt as the biospy report and imaging were not matching and hence the slides were send to senior pathologists for review who said biopsy in inconclusive. Hence, a repeat CT-guided biopsy was done in our institute which suggested possibility of “Angiomatous malformation.” Immunohistochemistry showed CD4 positivity on the endothelium and negative for cytokeratin. CT angiography was done to further know the nature of lesion which said that the lesion shows gradual enhancement and retention of contrast with no obvious feeder vessel seen. The patient underwent left postero lateral thoracotomy and excision of mass which was extra pulmonary as shown in [Figure 2]. As the patient had come with a chief complaint of repeated pleural effusion with ICD in situ for a month, additional pleurodesis was done on the left pleural space with talc. Postoperatively, there was no pleural fluid draining. ICD was removed on POD3. POD7 chest X-ray, no pleural effusion was seen with a complete expansion of the lung on ipsilateral side. On histopathological examination, grossly, it was a nodular cystic mass with smooth external surface. Cut section revealed solid cystic areas containing brownish material. Microscopy showed blood vessels, predominantly small with few larger vessels of variable wall thickening were also noticed, confirming it to be a hemangioma.
|Figure 1: Computed tomography scan (a) Axial view showing enhancing lesion at D3 level on the left paravertebral region. (b) Coronal view showing enhancing lesion at D3 level on the left paravertebral region with massive pleural effusion|
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|Figure 2: Intraoperative picture with mass in situ and postoperative specimen|
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| > Discussion|| |
Hemangiomas may be found all over the body but are rare in certain location. Pleuropulmonary hemangiomas are extremely rare. They may be congenital or may be as a part of Von-Hippel–Lindau disease. The epidemiology is rare, and the pathogenesis is unknown. After an exhaustive review of related literature, hemangiomas of the lung, chest wall (rib or muscle), and mediastinum have been reported,,,, and pleural hemangioma has been reported rarely., Of which, intrathoracic hemagioma with pleural effusion is very few.,,
There is a wide range of age at the time of diagnosis (26 days to 76 years), but 75% of cases are first discovered at 35 years of age or younger. There is no sex predilection.
Patients are usually asymptomatic or present with nonspecific complaints such as cough, chest pain, dyspnea, or other constitutional symptoms. Therefore, for the majority of patients, hemangiomas are discovered incidentally. It may present with recurrent straw-colored pleural effusion, just like in this case, masquerading as advanced malignancy. However, Nanaware et al. reported hemorrhagic pleural effusion as well.
The diagnosis of hemangioma depends primarily on imaging and final histopathological examination. CT enables accurate assessment of tumor morphology, location, extent, and pattern of enhancement and can be helpful in identifying tumor tissues and types. CT is more sensitive than a plain radiograph in detecting phleboliths, which are present in approximately 30% of hemangiomas. MRI enables the accurate characterization of tumor tissue and extent, including differentiation from the adjacent areas of inflammation. T1- and T2-weighted images typically reveal high-signal intensity in the mass. Dynamic MR images shows a similar appearance to hepatic hemangiomas; eccentric enhancement on early phase and filling in on delayed phase scans. FDG-PET findings have been mentioned only in hemangioma derived from the extremities. However, Sakurai et al. noticed that FDG accumulation in thoracic hemangioma is low and similar to extremity hemangioma. Although much literature is not available pertaining to the use of PET scan for thoracic hemangiomas, it is not recommended.
The treatment of hemangioma should be individualized, depending on the location of the tumor mass, the depth of its infiltration, and the age and cosmetic requirements of the patient. Comprehensive treatment strategies are recommended, including dry ice cryotherapy, radiotherapy, steroid treatment, sclerosing agent injection, vascular ligation, vascular embolism, and surgical excision.,
There are few case reports that we came across during our review of literature for pleuropulmonary hemangioma. Kuo et al. reported a case of rib hemangioma which was managed by the resection of the involved rib. Nanaware et al. and Sindhwani et al. in their cases of pleural hemangioma managed it by doing an open thoracotomy with excision of mass., For sclerosing hemangioma of the lungs, modern-day management is video-assisted thoracoscopic lobectomy whenever it is technically feasible. Even mediastinal hemangiomas are managed by thorascopic or open thoracotomy approach. Hence, it was noticed in these case reports that definitive diagnosis of the lesion would be difficult preoperatively until diagnostic excision of the mass is done either thoracoscopically or open thoracotomy method to confirm it.
We managed our patient by left posterolateral thoracotomy and excision of this paravertebral mass.
Hence, in patients presenting with recurrent pleural effusion, possibility of benign tumors such as hemangioma should be kept in mind and investigated. Hemangiomas, due to its nonspecific symptoms may be easily misdiagnosed. Its diagnosis depends primarily on imaging and final histopathological examination. Biopsy of these lesions can be dangerous as it can lead to torrential bleeding and is not recommended. However, in our case, imaging and biopsy were inconclusive and doubtful, until we completely excised this paravertebral mass.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]