Desmoplastic fibroma

Last updated
Desmoplastic fibroma
Specialty Oncology

In medicine, a desmoplastic fibroma is a low-grade malignant, locally aggressive, fibrous and rare tumor of the bone, affecting children and young adults, potentially resulting in cortical bone destruction. It usually affects craniofacial bones, mandible most frequently, long bones (metaphyseal femur, tibia, humerus). [1] The World Health Organization, 2020, reclassified these tumors as specific benign tumors in the category of fibroblastic and myofibroblastic tumors. [2]

Contents

Although it does not tend to metastatize, it has a high local recurrence and infiltrative growth. [3] Treatment consists in wide local excision to prevent otherwise frequent recurrences. [4] The role of radiotherapy and chemotherapy in this tumor still is unclear. [5]

Some cases have been described, in which an osteosarcoma has arisen from a desmoplastic fibroma. [6]

A famous occurrence of this particular form of the disease involved Italo-Australian Riccardo Torresan in 2011, with 18 cm of femur needing to be removed with the now widely recognized method of "aggressive curettage" being employed. [7]

See also

Related Research Articles

<span class="mw-page-title-main">Sarcoma</span> Type of malignant tumor (cancer)

A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates.

<span class="mw-page-title-main">Bone tumor</span> Medical condition

A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. There may be a lump, pain, or neurological signs from pressure. A bone tumor might present with a pathologic fracture. Other symptoms may include fatigue, fever, weight loss, anemia and nausea. Sometimes there are no symptoms and the tumour is found when investigating another problem.

<span class="mw-page-title-main">Osteosarcoma</span> Cancerous tumour in a bone

An osteosarcoma (OS) or osteogenic sarcoma (OGS) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin and that exhibits osteoblastic differentiation and produces malignant osteoid.

<span class="mw-page-title-main">Fibromatosis</span> Medical condition

The term fibromatosis refers to a group of soft tissue tumors which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence. It is classed by the World Health Organization as an intermediate soft tissue tumor related to the sarcoma family. Arthur Purdy Stout coined the term fibromatosis, in 1954.

<span class="mw-page-title-main">Fibrosarcoma</span> Medical condition

Fibrosarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcomas mainly arise in people between the ages of 25 and 79. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.

<span class="mw-page-title-main">Ameloblastoma</span> Medical condition

Ameloblastoma is a rare, benign or cancerous tumor of odontogenic epithelium much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusack. This type of odontogenic neoplasm was designated as an adamantinoma in 1885 by the French physician Louis-Charles Malassez. It was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.

<span class="mw-page-title-main">Desmoplastic small-round-cell tumor</span> Aggressive and rare cancer

Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. It is characterized by the EWS-WT1 fusion protein.

A peripheral ossifying fibroma, also known as ossifying fibrous epulis, is “a gingival nodule which is composed of a cellular fibroblastic connective tissue stroma which is associated with the formation of randomly dispersed foci of mineralised products, which consists of bone, cementum-like tissue, or a dystrophic calcification. The lesion is considered part of an ossifying fibroma, but that is usually considered to be a jaw tumor. Because of its overwhelming incidence on the gingiva, the condition is associated with two other diseases, though not because they occur together. Instead, the three are associated with each other because they appear frequently on gingiva: pyogenic granuloma and peripheral giant cell granuloma. Some researchers believe peripheral ossifying fibromas to be related to pyogenic fibromas and, in some instances, are the result of a pyogenic granuloma which has undergone fibrosis and calcification.

<span class="mw-page-title-main">Aggressive fibromatosis</span> Medical condition

Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). The tumors arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive and can cause life-threatening problems or even death when the tumors compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves. The condition is rarely fatal. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.

<span class="mw-page-title-main">Aneurysmal bone cyst</span> Medical condition

Aneurysmal bone cyst (ABC) is a non-cancerous bone tumor composed of multiple varying sizes of spaces in a bone which are filled with blood. The term is a misnomer, as the lesion is neither an aneurysm nor a cyst. It generally presents with pain and swelling in the affected bone. Pressure on neighbouring tissues may cause compression effects such as neurological symptoms.

<span class="mw-page-title-main">Ameloblastic fibroma</span> Medical condition

An ameloblastic fibroma is a fibroma of the ameloblastic tissue, that is, an odontogenic tumor arising from the enamel organ or dental lamina. It may be either truly neoplastic or merely hamartomatous. In neoplastic cases, it may be labeled an ameloblastic fibrosarcoma in accord with the terminological distinction that reserves the word fibroma for benign tumors and assigns the word fibrosarcoma to malignant ones. It is more common in the first and second decades of life, when odontogenesis is ongoing, than in later decades. In 50% of cases an unerupted tooth is involved.

Peripheral odontogenic fibroma(PFO) is a fibrous connective tissue mass that is exophytic and covered in surface epithelium that contains odontogenic epithelium. The World Health Organization (WHO) classifies peripheral odontogenic fibroma as a fibroblastic neoplasm with variable amounts of odontogenic epithelium that appears to be dormant. Dentine and/or cementum-like material may be present.

<span class="mw-page-title-main">Chondroblastoma</span> Medical condition

Chondroblastoma is a rare, benign, locally aggressive bone tumor that typically affects the epiphyses or apophyses of long bones. It is thought to arise from an outgrowth of immature cartilage cells (chondroblasts) from secondary ossification centers, originating from the epiphyseal plate or some remnant of it.

<span class="mw-page-title-main">Ewing sarcoma</span> Type of cancer

Ewing sarcoma is a type of pediatric cancer that forms in bone or soft tissue. Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture. The most common areas where it begins are the legs, pelvis, and chest wall. In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis. Complications may include a pleural effusion or paraplegia.

Collagenous fibroma, also known as desmoplastic fibroblastoma, is a slow-growing, deep-set, benign fibrous tumor, usually located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. The World Health Organization in 2020 reclassified desmoplastic fibroblastoma/collagenous fibroma as a specific benign tumor type within the broad category of fibroblastic and myofibroblastic tumors.

<span class="mw-page-title-main">Bone cyst</span> Medical condition

A bone cyst or geode is a cyst that forms in bone.

Osteofibrous dysplasia is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of bone.

Fibrocartilaginous mesenchymoma of bone (FCMB) is an extremely rare tumor first described in 1984. About 26 cases have been reported in literature, with patient ages spanning from 9 to 25 years, though a case in a male infant aged 1 year and 7 months has been reported. Quick growth and bulky size are remarkable features of this tumor.

A juvenile active ossifying fibroma is a benign fibro-osseous neoplasm composed of mixture of stroma and bone characterized by rapid and destructive growth.

Fibroblastic and myofibroblastic tumors (FMTs) develop from the mesenchymal stem cells which differentiate into fibroblasts and/or the myocytes/myoblasts that differentiate into muscle cells. FMTs are a heterogeneous group of soft tissue neoplasms. The World Health Organization (2020) defined tumors as being FMTs based on their morphology and, more importantly, newly discovered abnormalities in the expression levels of key gene products made by these tumors' neoplastic cells. Histopathologically, FMTs consist of neoplastic connective tissue cells which have differented into cells that have microscopic appearances resembling fibroblasts and/or myofibroblasts. The fibroblastic cells are characterized as spindle-shaped cells with inconspicuous nucleoli that express vimentin, an intracellular protein typically found in mesenchymal cells, and CD34, a cell surface membrane glycoprotein. Myofibroblastic cells are plumper with more abundant cytoplasm and more prominent nucleoli; they express smooth muscle marker proteins such as smooth muscle actins, desmin, and caldesmon. The World Health Organization further classified FMTs into four tumor forms based on their varying levels of aggressiveness: benign, intermediate, intermediate, and malignant.

References

  1. "Desmoplastic Firboma".
  2. Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC   8167394 . PMID   33179614.
  3. Schneider, M.; Zimmermann, A. C.; Depprich, R. A.; Kübler, N. R.; Engers, R.; Naujoks, C. D.; Handschel, J. (2009). "Desmoplastic fibroma of the mandible - review of the literature and presentation of a rare case". Head & Face Medicine. 5: 25. doi: 10.1186/1746-160X-5-25 . PMC   2787487 . PMID   19930688.
  4. "Desmoplastic fibroma".
  5. "Desmoplastic fibroma".
  6. Abdelwahab, Ibrahim Fikry; Klein, Michael J.; Hermann, George; Steiner, German C.; Yang, David C. (2002). "Osteosarcoma Arising in a Desmoplastic Fibroma of the Proximal Tibia". American Journal of Roentgenology. 178 (3): 613–615. doi:10.2214/ajr.178.3.1780613. PMID   11856684.
  7. "Desmoplastic Fibroma - BONETUMOR.ORG".