Survival Following Treatment of a Cardiac Malignant Fibrous Histiocytoma

Schena, Stefano; Caniglia, Alessandro; Agnino, Alfonso; Caruso, G.; Ferlan, G.

doi:10.1378/chest.118.1.271

Cited by 15 publications

(14 citation statements)

References 6 publications

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“…However, there are some reports about successful favourable response with combined treatment particularly in patients with high tumor mitotic rate [4,5]. …”

Section: Resultsmentioning

confidence: 99%

"Malignant" mitral stenosis

Auer¹,

Berent²,

Gurtner³

2012

J Cardiothorac Surg

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Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor is extremely rare and frequently associated with poor prognosis. We report a case of a 59-year-old man with a history of grade 3 malignant fibrous histiocytoma on his left tigh treated by limb-sparing surgery 17 months earlier, who was admitted with 10-days of worsening dyspnea. Imaging revealed a left atrial mass protruding through the mitral valve that resulted in severe mitral stenosis. Biopsy confirmed metastasis of malignant fibrous histiocytoma.

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“…However, there are some reports about successful favourable response with combined treatment particularly in patients with high tumor mitotic rate [4,5]. …”

Section: Resultsmentioning

confidence: 99%

"Malignant" mitral stenosis

Auer¹,

Berent²,

Gurtner³

2012

J Cardiothorac Surg

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“…In addition, surgical resection is necessary to ameliorate symptoms and to obtain a histologic diagnosis despite a high incidence of local recurrence and distant metastasis [10]. Due to the aggressive nature of this tumor, the average survival time is less than one year [13]. Orthotopic heart transplantation (OHT) has been reported, but the evidence of a clear benefit for MFH is limited [14].…”

Section: Discussionmentioning

confidence: 99%

“…The diagnostic criteria for MFH is a continuous subject of debate amongst pathologists [13]. In fact, the "malignant fibrous histiocytoma" category was removed from the 2013 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone and replaced with an "undifferentiated/unclassified sarcoma" category that reflects the lack of distinguishing histologic, immunohistochemical, or genetic features [18].…”

Section: Discussionmentioning

confidence: 99%

A Unique Case of Rapidly Progressive Diffuse Pulmonary Nodules

Weiler¹,

Puopolo²,

Newmarch³

et al. 2017

Pulm Crit Care Med

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The following case report describes a unique presentation of primary malignant fibrous histiocytoma (MFH) originating in the right ventricle. A 71-year-old male presented to the emergency department with tachycardia, dyspnea, tachypnea, and left leg pitting edema, seven weeks following a right total hip arthroplasty. Six weeks prior to presentation, the patient was diagnosed with pulmonary embolism and five pulmonary nodules based on computed tomography (CT) angiography. Laboratory studies at this time indicated anemia of chronic disease, consistent with results from six weeks earlier. Broncho-alveolar lavage (BAL) cytology suggested adenocarcinoma. CT angiography demonstrated a dramatic increase in the amount and extent of the pulmonary nodules previously noted in addition to an unresolved embolus of the right upper lobe. CT-guided biopsy was performed and confirmed a definitive diagnosis of non-small cell lung cancer. Two days after CT-guided biopsy, the patient developed acute hypotension and hypoxic respiratory failure rapidly progressing to asystole and subsequent death. Autopsy revealed a primary intra-cavitary malignant fibrous histiocytoma in the right ventricle, and multiple nodular tumours throughout the lung parenchyma with chest wall invasion. These findings allowed for a revision of the diagnosis and clinical-pathological picture. This case illustrates the limitations of radiological imaging in visualizing vascular structures, and the continued importance of autopsy in achieving a complete and correct pathological diagnosis. Moreover, this case draws light to the unclear diagnostic criteria for MFH and lack of effective treatment options. The longest documented survival of MFH is eight years, which was achieved with orthotopic cardiac transplantation.

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“…3 There are few single case reports of successful chemotherapy and radiotherapy. 4 In fact, surgical resection is the only effective therapy for MFH. 1 Given that many articles report the need for frequent and repeated resection of local recurrence in individual patients, MFH should be removed extensively, including all layers of the LA.…”

Section: Discussionmentioning

confidence: 99%