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Pierre-Marie-Bamberger syndrome – A paraneoplastic syndrome of solitary fibrous tumour of the pleura

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1 Resident, Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal

2 Graduate Assistant, Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal

3 Senior Graduate Assistant, Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Internal Medicine Clinic, Faculty of Medicine, Univers, Coimbra, Portugal

4 Senior Graduate Assistant and Head of Department, Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Internal Medicine Clinic, Faculty of Medicine, Univers, Coimbra, Portugal

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Sara Neves Sintra

Praceta Prof. Mota Pinto, Coimbra 3000-075,

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Article ID: 100987Z01SS2018

doi:10.5348/100987Z01SS2018CL

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Sintra SN, Filipe C, Simão A, Carvalho A. Pierre- Marie-Bamberger syndrome – A paraneoplastic syndrome of solitary fibrous tumour of the pleura. Int J Case Rep Images 2018;9:100987Z01SS2018.

ABSTRACT


No Abstract

Keywords: Pleura, Solitary fibrous tumour, Secondary hypertrophic osteoarthropathy

Case Report


The authors report a case of a 75-year-old woman who presented to the Emergency Department with dyspnoea, productive cough, vomiting and unquantified weight loss. She denied fever, night sweats or other symptoms. She had a past history of dementia, dyslipidaemia and right total hip replacement. No relevant family history was known. On physical examination, the patient was fully awake, relatively mobile and disoriented to place and time. Her vital signs were within normal limits with an oxygen saturation of 94% on room air. Lung examination revealed absence of breath sounds over the right hemithorax with increased dullness to percussion. Bilateral pitting oedema extending to the joints was seen in the lower extremities. She complained of pain while palpating both femurs. Digital clubbing was also notable (Figure 1A). Laboratory findings were as follows: haemoglobin 13.7 g/dL (normal, 12–15), mean corpuscular volume 82.6 fL (normal, 83–101), leucocytes 13.7x109/L (normal, 4–10), platelets 310x109/L (normal, 150–400), lactate dehydrogenase 442 U/L (normal, 125– 220), C-reactive protein 4.97 (normal, 0–0.5); with no other relevant findings. Chest radiograph report described a significant right diaphragmatic elevation (Figure 1B). Thoracic and abdominal computed tomography (CT) showed a 17.4x14x19.5 cm heterogeneous mass occupying almost the whole right hemithorax, with areas of necrosis, causing right main and lobar bronchial deviation; small right pleural effusion and liver cysts (Figure 1C). Frontal pelvis radiograph showed multilayered periostitis involving both femoral metaphysis and diaphysis (Figure 2). Bronchoscopy was performed and revealed strictures involving the right main and superior lobar bronchi, hindering the progression of the bronchoscope. Transbronchial biopsies were done through the edematous right superior lobar bronchus and revealed basal cell hyperplasia, without neoplastic cells.

Percutaneous CT-guided biopsy was then performed. Histopathologic examination revealed a fibrous tumour, with negative immunohistochemistry for keratin, S-100 protein, alpha-smooth muscle actin and positivity for CD34, vimentin, bcl-2 and for Ki-67 in 10% of cells in hypercellular areas.

The hospital stay was complicated with recurrent hip prosthesis dislocation, which led to severe functional restriction. As physical rehabilitation was nearly impossible, multidisciplinary treatment decision was to provide palliative care.The patient died of hospital acquired pneumonia seventy-two days after diagnosis.

Figure 1: (A) Digital clubbing, (B) Anteroposterior chest radiograph image demonstrates a right hemithorax opacification with contralateral mediastinal shift, (C) Coronal volumerendered CT image showing a 17.4x14x19.5 cm heterogeneous mass in the right hemithorax, with areas of necrosis.

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Figure 2: Frontal pelvis radiograph shows right hip prosthesis dislocation and multilayered periostitis involving the femoral metaphysis and diaphysis.

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Discussion


Solitary fibrous tumours (SFT) are relatively rare mesenchymal tumours of fibroblastic origin that can affect any organ [1]. SFT of the pleura (SFTP) account for less than 5% of all pleural tumours [2]. They affect adults in their sixth and seventh decades of life and typically present as incidental asymptomatic slow-growing masses [1]. Larger tumours are more likely to present with symptoms, like cough, dyspnoea, pleuritic chest pain and haemoptysis due to compression of a bronchus [1]. SFTP may be associated with secondary hypertrophic osteoarthropathy also known as Pierre-Marie-Bamberger syndrome, characterized by digital clubbing, periostitis and arthritis, which is thought to be related to increased production of hyaluronic acid by tumour cells [1],[3]. Although it is most commonly associated with non-small cell lung cancer, specifically adenocarcinoma (reported in 0.7–17%), a percentage of up to 20% of SFTP present with this paraneoplastic syndrome [4]. Most of SFTP are benign, although some behave aggressively [1]. Surgical resection via thoracotomy is the mainstay of treatment for most localized SFTP [1]. Resection of giant-sized tumours, such as the one described herein, can be more challenging, due to poor exposure and visualisation, as well as significant blood supply to the tumour [1],[2]. Treatment of secondary hypertrophic osteoarthropathy is best achieved by definitive treatment of the primary pathology, whenever possible [3],[4].

Conclusion


Pierre-Marie-Bamberger syndrome is a rare paraneoplastic syndrome, which is rarely associated with SFTP. In a patient with clinical diagnosis of clubbing and joint or bone pain, attention must be directed to chest imaging, even if asymptomatic. Definitive clinical management of this paraneoplastic syndrome is aimed at treating the underlying cause, whenever possible.

REFERENCES


1.

Raafat E, Karunasiri D, Kamangar N. Solitary fibrous tumour of the pleura presenting as a giant intrathoracic mass. BMJ Case Rep 2017 Jun 30;2017. [CrossRef] [Pubmed]   Back to citation no. 1  

2.

Crnjac A, Veingerl B, Vidovic D, Kavalar R, Hojski A. Giant solitary fibrous tumour of the pleura. Case report and review of the literature. Radiol Oncol 2015 Nov 27;49(4):395–401. [CrossRef] [Pubmed]   Back to citation no. 1  

3.

Boyer-Duck E, Dajer-Fadel WL, Hernández-Arenas LÁ, Macías-Morales MP, Rodríguez-Gómez A, Romo-Aguirre C. Pierre-Marie-Bamberger syndrome and solitary fibrous tumor: A rare association. Asian Cardiovasc Thorac Ann 2018 Feb;26(2):154–7. [CrossRef] [Pubmed]   Back to citation no. 1  

4.

Chakraborty RK, Sharma S. Secondary Hypertrophic Osteoarthropathy. Treasure Island (FL): StatPearls Publishing; 2018 Jan.   Back to citation no. 1  

SUPPORTING INFORMATION


Author Contributions

Sara Neves Sintra - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Carlos Filipe - Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Adélia Simão - Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published

Armando Carvalho - Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published

Guarantor of Submission

The corresponding author is the guarantor of submission.

Source of Support

None

Consent Statement

Written informed consent was obtained from the patient for publication of this clinical image.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Conflict of Interest

Authors declare no conflict of interest.

Copyright

© 2018 Sara Neves Sintra et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.


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