Pierre-Marie-Bamberger syndrome and solitary fibrous tumor: a rare association

Boyer-Duck, Estefanía; Dajer-Fadel, Walid Leonardo; Hernandez-Arenas, Luis Angel; Macías-Morales, Marco Polo; Rodríguez-Gómez, Adriana; Romo-Aguirre, Catalina

doi:10.1177/0218492318757042

Cited by 14 publications

(8 citation statements)

References 6 publications

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“…DPS occurs when a large SFT ectopically excretes Insulin-like growth factor 2 (IGF-2), resulting in a type of hyperinsulinemic hypoglycemia [6]. DPS was first exclusively described in 1930 by Doege and Potter as a non-islet cell tumor hypoglycemia [7].…”

Section: Introductionmentioning

confidence: 99%

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review

Solsi

Pho

Shojaie

et al. 2020

Cureus

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Solitary fibrous tumors (SFT) represent a unique subset of mostly benign heterogeneous tumors with mesenchymal cell origins. These tumors have been reported in the past as being mostly indolent, with a slowly evolving clinical course and low potential for malignancy. Although found systemically, the incidence of SFT arising intrathoracically, from the pleura of the lung, is relatively poorly documented in the medical literature. SFT is a rare phenomenon, but in even rarer circumstances, these tumors are associated with distinctive paraneoplastic syndromes, such as Pierre-Marie-Bamberger syndrome (PMBS) and Doege-Potter syndrome (DPS). PMBS presents as digital clubbing and hypertrophic pulmonary osteoarthropathy. DPS has been characterized as a non-islet cell tumor hypoglycemia due to the ectopic secretion of insulin-like growth factor 2 (IGF-2), a pattern seen in fewer than 5% of cases of SFT. Treatment is typically through surgical resection. In our research of the medical literature, we found only very few cases in which the association with SFT and both paraneoplastic syndromes were described. Here, we report an uncommon case of a 68-year-old male patient found to have an incidental right hemithoracic tumor with digital clubbing and intermittent severe episodes of fasting hypoglycemia after initially presenting with a syncopal episode.

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Section: Introductionmentioning

confidence: 99%

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review

Solsi

Pho

Shojaie

et al. 2020

Cureus

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“…In large series of cases, the prevalence of lung neoplasm in patients with hypertrophic osteoarthropathy varied between 4% and 32%. [8] In a study that enrolled 81 patients with bronchopulmonary malignant tumors from our country, the authors found that 31.6% of the patients had a complete or incomplete Pierre Marie–Bamberger syndrome. [9] It is considered that the prevalence is higher in patients with nonsmall-cell lung tumors than in those with small-cell lung carcinoma probably due to a longer survival and a better prognosis of the former.…”

Section: Discussionmentioning

confidence: 99%

Nociceptive pain unmasking a serious pathology – paraneoplastic hypertrophic osteoarthropathy

et al. 2019

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Rationale: Hypertrophic osteoarthropathy, also named Pierre Marie–Bamberger syndrome, represents a rare medical condition that may be considered either a primary or a secondary disease, and lung malignancies are responsible for more than two-thirds of the cases with secondary forms of the disease. Patient concerns: We present the case of a 41-year-old man referred to our Neurology Department for pain that was considered secondary to cervical disc protrusions. The neurologic examination was normal. However, the general examination showed digital clubbing, right lateral cervical adenopathy, and pachydermia. The radiographic examinations of the upper and lower limbs depicted osseous abnormalities typical for periostosis, and the computed tomography of the thorax showed the presence of a mass lesion in the right upper pulmonary lobe. High values of vascular endothelial growth factor were also found. The patient was admitted to the Pneumology Clinic, where biopsy was performed from the lateral cervical adenopathy. Diagnoses: The anatomopathological examination revealed multiple neoplastic infiltrates suggestive of adenocarcinoma metastasis. Based on the clinical examination and radiological and histologic findings, the diagnosis of pulmonary adenocarcinoma with lymph nodes metastases and paraneoplastic hypertrophic osteoarthropathy was established. Interventions: The patient received treatment with nonsteroidal antiinflammatory drugs and opiate analgesics that relieved the pain. Outcomes: The patient was referred to the Oncology Department for further treatment of the primary pathology. He received different types of chemotherapeutics, immunotherapy, and radiotherapy. However, despite all therapeutic measures, the disease rapidly progressed and the patient died 9 months later. Lessons: This is an interesting case of a patient with an overlooked pathology, which was refereed to our clinic for further investigations of a pain that was considered neuropathic, secondary to small cervical protrusions. Conversely, the pain proved to be nociceptive and Pierre Marie–Bamberger syndrome was the positive diagnosis in our patient, as it can be associated with numerous diseases, especially of neoplastic origin.

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“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Discussionmentioning

confidence: 99%

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

Sintra¹

2018

Int J Case Rep Images

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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 13.7x10 9 /L (normal, 4-10), platelets 310x10 9 /L (normal, 150-400), lactate dehydrogenase 442 U/L (normal, 125-220), C-reactive protein 4.97

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Pierre-Marie-Bamberger syndrome and solitary fibrous tumor: a rare association

Cited by 14 publications

References 6 publications

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review

Nociceptive pain unmasking a serious pathology – paraneoplastic hypertrophic osteoarthropathy

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

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