[Maffucci's syndrome with giant tumor of the thoracic wall]

Marcin Strzałka1, Włodzimierz Drozdz, Jan Kulawik

  • 1II Katedra Chirurgii Ogólnej Collegium Medicum Uniwersytetu Jagiellońskiego, 31-501 Kraków, ul. Kopernika 21.

Przeglad Lekarski
|December 19, 2003
PubMed

Insights

Maffucci's syndrome, a rare mesodermal dysplasia, involves hemangiomas and enchondromas. This case highlights a unique giant thorax tumor, a rare complication of this condition.

Area of Science:

  • Medical Genetics
  • Oncology
  • Pathology

Background:

  • Maffucci's syndrome is a rare, nonhereditary disorder characterized by mesodermal dysplasia, presenting as multiple soft tissue hemangiomas and enchondromas.
  • The condition is associated with an increased risk of various tumors, predominantly of mesodermal origin, including malignant neoplasms like chondrosarcoma and benign adenomas.

Observation:

  • This report details a rare case of Maffucci's syndrome in a 26-year-old female.
  • The patient presented with an unusually large thorax tumor, a manifestation not commonly documented in existing literature.

Findings:

  • Histopathological examination revealed the giant thorax tumor to be composed of fibroadenoma and canalicular adenoma.
  • This specific tumor composition represents a unique finding in the context of Maffucci's syndrome.

Implications:

  • This case expands the known spectrum of tumors associated with Maffucci's syndrome.
  • Further research into the tumorigenesis of Maffucci's syndrome may elucidate novel therapeutic targets.

Related Concept Videos

Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
Appendicitis01:19

Appendicitis

Appendicitis is an acute inflammatory condition of the vermiform appendix, most commonly caused by obstruction of its lumen. The appendix is a narrow, blind-ended pouch that extends from the cecum, making it particularly prone to obstruction. Causes include fecaliths, lymphoid hyperplasia (often after viral infections), parasites, tumors, or foreign bodies. This obstruction initiates a cascade of pathological changes.Luminal Obstruction and Early InflammationAfter obstruction, normal mucosal...
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
Flail Chest-I01:24

Flail Chest-I

Overview of Flail Chest
Flail chest is a severe and potentially life-threatening condition characterized by the fracture of three or more adjacent ribs in multiple places. It is most commonly caused by direct impacts and trauma, such as motor vehicle accidents or injuries from a steering wheel impact. It can also occur due to falls in elderly individuals with osteoporosis, or assaults involving sharp objects.
Pathophysiology
The pathophysiology of flail chest is complex, involving fractures of...