| Elastofibroma dorsi | |
|---|---|
 | |
| Bilateral elastofibroma dorsi in native computed tomography: left image axial in prone position (for biopsy), right image oblique coronal view. |
Elastofibroma dorsi is an ill-defined fibroelastic tumor-like condition made up of enlarged and irregular elastic fibers. [1] [2] The World Health Organization, 2020, has classified elastofibroma tumors as one specific type of the fibroblastic and myofibroblastic tumors. [3]
Patient will present with a slow growing, deep-seated, firm mass, often presenting bilaterally. There may be pain or tenderness, but this is rare. [1] [2] [4]
When only present on one side, they are more often present on the right side. [5]
There are several theories about origin:
By computed tomography, there is a poorly circumscribed, heterogeneous soft tissue mass, with a signal intensity similar to skeletal muscle. The fact that the lesion may be bilateral, helps eliminate a sarcoma from further consideration. [6] At US, elastofibromas are depicted deep to the musculature as a multilayered pattern of hypoechoic linear areas of fat deposition intermixed with echogenic fibroelastic tissue. [7] The mass often protrudes from the subscapular region upon shoulder abduction, allowing better delineation of the finding. [8]
In general, the tumor is an ill-defined, nonencapsulated, rubbery, and firm, white lesion with interspersed fat. The tumors can be quite large (up to 20 cm), although most are around 5 cm. [4]
By microscopie view, there is an admixture of heavy dense bands of collagenous tissue dissected by fat and abnormal elastic fibers. The elastic fibers are often quite large and are easily identified. The elastic fibers are coarse, thick, and darkly eosinophilic, often fragmented into globules, creating a "string of pearls" or "pipe cleaner" appearance. Because of degeneration, the elastic fibers will appear as globules with a serrated or "prickled" edge. [4]
The elastic fibers will be highlighted by a Weigert or von Gieson elastic stains. [9]
Given the anatomic site, a spindle cell lipoma, nuchal-type fibroma and fibromatosis colli are all included in the differential diagnosis. [10]
Simple excision is the treatment of choice, although given the large size, bleeding into the space can be a potential complication. Isolated recurrences may be seen, but there is no malignant potential. [2] [4] [11]
This is a very rare phenomenon (< 0.001% of soft tissue tumors), usually presenting in elderly patients (>50 years of age), and more commonly in women than men (5:1). There is an increased frequency in Okinawa, Japan, but this may be a reporting bias. The tumor develops very specifically in the subscapular or infrascapular area, deep to the muscle, sometimes even attached to periosteum of ribs. It is usually between the shoulder blade and the lower neck, with rare tumors reported in the chest wall. [1] [2] [4]
The scapula, also known as the shoulder blade, is the bone that connects the humerus with the clavicle. Like their connected bones, the scapulae are paired, with each scapula on either side of the body being roughly a mirror image of the other. The name derives from the Classical Latin word for trowel or small shovel, which it was thought to resemble.
The latissimus dorsi is a large, flat muscle on the back that stretches to the sides, behind the arm, and is partly covered by the trapezius on the back near the midline. The word latissimus dorsi comes from Latin and means "broadest [muscle] of the back", from "latissimus" and "dorsum". The pair of muscles are commonly known as "lats", especially among bodybuilders. The latissimus dorsi is the largest muscle in the upper body.
A soft-tissue sarcoma (STS) is a malignant tumor, a type of cancer, that develops in soft tissue. A soft-tissue sarcoma is often a painless mass that grows slowly over months or years. They may be superficial or deep-seated. Any such unexplained mass must be diagnosed by biopsy. Treatment may include surgery, radiotherapy, chemotherapy, and targeted drug therapy. Bone sarcomas are the other class of sarcomas.
A hamartoma is a mostly benign, local malformation of cells that resembles a neoplasm of local tissue but is usually due to an overgrowth of multiple aberrant cells, with a basis in a systemic genetic condition, rather than a growth descended from a single mutated cell (monoclonality), as would typically define a benign neoplasm/tumor. Despite this, many hamartomas are found to have clonal chromosomal aberrations that are acquired through somatic mutations, and on this basis the term hamartoma is sometimes considered synonymous with neoplasm. Hamartomas are by definition benign, slow-growing or self-limiting, though the underlying condition may still predispose the individual towards malignancies.
A vascular tumor is a tumor of vascular origin; a soft tissue growth that can be either benign or malignant, formed from blood vessels or lymph vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, Kaposi's sarcomas, angiosarcomas, and hemangioblastomas. An angioma refers to any type of benign vascular tumor.
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.
Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, is characterized by the World Health Organization (WHO), 2020, as a rare, poorly differentiated neoplasm, i.e. an abnormal growth of cells that have an unclear identity and/or cell of origin. WHO classified it as one of the undifferentiated/unclassified sarcomas in the category of tumors of uncertain differentiation. Sarcomas are cancers known or thought to derive from mesenchymal stem cells that typically develop in bone, muscle, fat, blood vessels, lymphatic vessels, tendons, and ligaments. More than 70 sarcoma subtypes have been described. The UPS subtype of these sarcomas consists of tumor cells that are poorly differentiated and may appear as spindle-shaped cells, histiocytes, and giant cells. UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved.
Primary tumors of the heart are extremely rare tumors that arise from the normal tissues that make up the heart. The incidence of primary cardiac tumors has been found to be approximately 0.02%. This is in contrast to secondary tumors of the heart, which are typically either metastatic from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues. Metastatic tumors to the heart are about 20 times more common than primary cardiac tumors.
A myxoma is a rare benign tumor of the heart. Myxomata are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium near the valve of the fossa ovalis. Myxomata may also develop in the other heart chambers. The tumor is derived from multipotent mesenchymal cells. Cardiac myxoma can affect adults between 30 and 60 years of age.
Kaposi's sarcoma (KS) is a type of cancer that can form masses in the skin, in lymph nodes, in the mouth, or in other organs. The skin lesions are usually painless, purple and may be flat or raised. Lesions can occur singly, multiply in a limited area, or may be widespread. Depending on the sub-type of disease and level of immune suppression, KS may worsen either gradually or quickly. Except for Classical KS where there is generally no immune suppression, KS is caused by a combination of immune suppression and infection by Human herpesvirus 8.
Clear cell sarcoma is a rare form of cancer called a sarcoma. It is known to occur mainly in the soft tissues and dermis. Rare forms were thought to occur in the gastrointestinal tract before they were discovered to be different and redesignated as gastrointestinal neuroectodermal tumors.
Mucinous cystadenocarcinoma of the lung (MCACL) is a very rare malignant mucus-producing neoplasm arising from the uncontrolled growth of transformed epithelial cells originating in lung tissue.
Nuchal-type fibroma is a rare benign proliferation involving the dermis and subcutaneous tissues, that is a collection of dense, hypocellular bundles of collagen with entrapped adipocytes and increased numbers of small nerves. It is no longer called a nuchal fibroma, but instead a "nuchal-type fibroma" since it develops in other anatomic sites. There is no known etiology. The World Health Organization in 2020 classified nuchal fibromas as a specific tumor form in the category of benign fibroblastic and myofibroblastic tumors.
Hyperthermic intrathoracic chemotherapy (HITOC) is part of a surgical strategy employed in the treatment of various pleural malignancies. The pleura in this situation could be considered to include the surface linings of the chest wall, lungs, mediastinum, and diaphragm. HITOC is the chest counterpart of HIPEC. Traditionally used in the treatment of malignant mesothelioma, a primary malignancy of the pleura, this modality has recently been evaluated in the treatment of secondary pleural malignancies.
In medicine, vein graft failure (VGF) is a condition in which vein grafts, which are used as alternative conduits in bypass surgeries, get occluded.
Vertebral hemangiomas or haemangiomas (VHs) are a common vascular lesion found within the vertebral body of the thoracic and lumbar spine. These are predominantly benign lesions that are often found incidentally during radiology studies for other indications and can involve one or multiple vertebrae. Vertebral hemangiomas are a common etiology estimated to be found in 10-12% of humans at autopsy. They are benign in nature and frequently asymptomatic. Symptoms, if they do occur, are usually related to large hemangiomas, trauma, the hormonal and hemodynamic changes of pregnancy (causing intra-spinal bleeding), or osseous expansion and extra-osseous extension into surround soft tissues or epidural region of the spinal canal.
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.
Ischemic fasciities (IF), also termed atypical decubital fibroplasia or decubital ischemic fasciitis, is a rare pseudosarcomatous tumor. It was first described by E. A. Montgomery et al. in 1992. This tumor typically forms in the subcutaneous tissues that overlie bony protuberances such as a hip in individuals who are debilitated and bed-ridden.
Cellular angiofibroma (CAF) is a rare, benign tumor of superficial soft tissues that was first described by M. R. Nucci et al. in 1997. These tumors occur predominantly in the distal parts of the female and male reproductive systems, i.e. in the vulva-vaginal and inguinal-scrotal areas, respectively, or, less commonly, in various other superficial soft tissue areas throughout the body. CAF tumors develop exclusively in adults who typically are more than 30 years old.
Low-grade myofibroblastic sarcoma (LGMS) is a subtype of the malignant sarcomas. As it is currently recognized, LGMS was first described as a rare, atypical myofibroblastic tumor by Mentzel et al. in 1998. Myofibroblastic sarcomas had been divided into low-grade myofibroblastic sarcomas, intermediate‐grade myofibroblasic sarcomas, i.e. IGMS, and high‐grade myofibroblasic sarcomas, i.e. HGMS based on their microscopic morphological, immunophenotypic, and malignancy features. LGMS and IGMS are now classified together by the World Health Organization (WHO), 2020, in the category of intermediate fibroblastic and myofibroblastic tumors. WHO, 2020, classifies HGMS as a soft tissue tumor in the category of tumors of uncertain differentiation. This article follows the WHO classification: here, LGMS includes IGMS but not HGMS which is a more aggressive and metastasizing tumor than LGMS and consists of cells of uncertain origin.
Lester D. R. Thompson; Bruce M. Wenig (2011). Diagnostic Pathology: Head and Neck: Published by Amirsys. Hagerstown, MD: Lippincott Williams & Wilkins. pp. 8:30–31. ISBN 978-1-931884-61-7.