67 terms

path b19 soft tissue

Genetic syndromes
A minority in soft tissue tumors
Li-Fraumeni syndrome
Soft tissue SARCOMA; defective p53 gene. These patients are prone to different malignancies
Neurofibromatosis type I
Maligant peripheral nerve sheath tumor, neurofibromas
Osler-Weber Rendu
Telangiectasia (superficial - skin, mucus membranes) and arteriovenus malformations (organs). Arterioles directly connect to venules.
Gardner syndrome
Fibromatosis/ desmoids tumors. Multiple colonic polyps, associated with colonic adenmoas
Tumors of adipose tissue
Benign, most common soft tissue tumor (STT) characteristic cytogenetic abnormalities in the subcutaneous tissue of the prox. Extremities and trunk. Well encapsulated and consists of mature adipose tissue; Painless,soft, and mobile and cured by excision
Lipoma subtypes
Conventional lipoma
Discussed above; abnormal chromosomal rearrangements
Spindle cell lipoma
Abnormal chromosomal rearrangements
Pleomorphic lipoma
Malignant tumor of adipose tissue. MOST common Sarcoma of adulthood located deep in the soft tissue, Prox. Extremities and retroperoneum. Masses are LARGE and bulky. 3 grades: well differentiated Grade I; Mixoid - Grade II; Pleomorphic - Grade III. May recur after excision
Tumor like conditions of adipose tissue
Fat necrosis
Can occur in the pancreas. In other areas it is painless palpable mass. Skin thickening or retraction; mammographic density or calcification (common in breasts); prior history of trauma. Major clinical significance is confusion with malignancy
Fine needle aspiration
Use it to withdraw some biopsy material; ethanol fixed slide, air dried slide;
Fibrous tumors
Non neoplastic, secondary to trauma(physical, ischemic), grow rapidly. Histo: hypercellular with plump fibroblast and mitosis. Proliferative lesions that can be confused with cancer.
Fibrous Tumor-like conditions
Non neoplastic, secondary to trauma(physical, ischemic), grow rapidly. Histo: hypercellular with plump fibroblast and mitosis. Proliferative lesions that can be confused with cancer.
Pseudosarcomatous proliferation
Nodular fasciitis/ pseudosarcomatous fasciitis
Most common reactive pseudosarcoma in adults; chest, arms, back; history is several weeks. Solitary, rapidly growing, painful mass that preceding truma. It has a decent capsule and zonation, the central zone is eosinophilic and the peripheral zone is cellular. Looks malignant like spindle cell carcinoma (DDx); Histo: located deep in dermis, subcutis, muscle, poorly defined borders. Immature fibroblasts/myofibroblasts. Pleomorphic, conspicuous nuclei, abundant mitotic figures with extravasated blood and lymphocytes
Myositis ossificans
Adolescents. Reactive fibroblastic proliferation with calcifying mass in soft tissue or metaplastic bone tissue. Common in muscles of proximal extremities. It is a 3-6 cm well demarcated lesion with history of previous trauma (>50).
Looks like extraskeletal osteosarcoma (DDx)
Tumor like conditions/ proliferation of fibroblasts
Dupuytren contracture
Thickening of palmar skin due to proliferation of fibroblasts between picky and ring finger, slow progression. Has functional problems extending fingers and often bilateral
Plantar fibromatosis
Doesn't cause much functional problems because fibroblast thickening on sole of foot and often unilateral
Peyronie disease
Fibrous penile disease leading to deformities and preventing intercourse, usually thickened on the dorsum
Deep seated
Desmoid tumors
M=F of any age but often in found in TEENs. Well differentiated fibroblasts; they don't metastasize; these are low grade malignant tumors and can encase the abdominal organs and cause fibrosis and constriction. Can be disfiguring, disabling, painful and responds to chemo like malignant tumors do. This thing is proliferative, benign, and malignant! Adequate excision is usually curative; recur locally if incompletely excised.
3 types
Extra abdominal - shoulder, chest wall, back thigh MALES = FEMALES
Abdominal - musculo-aponeurotic structures of abdominal wall found in women after pregnancy
Intra-abdominal - mesenteric or pelvic wall and is associated with Gardner's syndrome (familial adenomatous polyposis)
Anything benign cannot
Metastasize (obviously)
Malignant tumors of fibrous tissue
Malignant tumors of fibroblasts. SEE BONE section for more INFO. It is unencapsulated/ hypercelluar with a Herringbone pattern. Infiltrative + pleomorphic . Aggressive, metastatic, and often reoccur. If it's very malignant and high grade, you don't see a good herring bone so you have to do immunohistochemistry. IN this case there is usually hemorrhage, necrosis, and mitosis.
Fibrohistocytic tumors
Descriptive term for cells that look like fibroblast and macrophages (histocytes). They are in reality, fibroblastic in origin.
Benign fibrous histiocytoma/ dermatofibroma
Middle aged FEMALES' legs are affected. Painless, slow growing (<1cm), and can be pigmented. Confused with seborrhic keratosis or melanoma. DOWNWARD dimpling upon compression lateral compression. Benign spindle cells with overlying epithelial hyperplasia
Malignant fibrous histiocytoma
Renamed to Fibrosarcoma b/c this description is an oxymoronic paradox (paradoxical oxymoron?) it's just a contradiction
Tumors of skeletal muscles
Benign rhabdomyoma
Found in cardiac cells associated with tuberous sclerosis
Malignant rhabdomyosarcoma
<20 people. Most common Soft tissue tumor of childhood. Found in head, neck, and genitourinary tract. Diagnostic is tadpole cell or strap cell; Immunohistochemistry: DESMIN, myogen, MYOD1 markers
Pleomorphic rhabdomyosarcoma
RARE, grade III, and located deep in soft tissues. They are numerous, large, bizarre, and multinucleated.
Alveolar rhabdomyosarcoma
EARLY adolescents. Grade II. 20% of rhabdomyosarcoma found in, deep muscles of the extremities. Fibrous septae resembling alveoli
Embryonal rhabdomyosarcoma
CHILDREN < 10Grade I, 60% of rhyabdomyosarcomas. Found in head or genital region
Sarcoma botryoides
Part of embryonal rhabdomyosarcoma that resembles bunches of grapes.
Tumors of smooth muscles
Benign leiomyomas
Malignant leiomyosarcoma
20% of tissue sarcomas. Found in the deep soft tissues of the extremities or retropreitoneum. NOT COMMON in Uterus. Pain, firm, bulky mass with malignant smooth muscle spindle cells; IHC: Smooth muscle Actin (SMA); DESMIN
Glomus Tumor/ glomangioma
Benign painful tumors of the distal digits underneath the fingernails. Derived from MODIFIED smooth muscle cells
Other tumors
Synovial Sarcoma
Soft tissue tumor growing around the joint, knee, or thigh. Only 10% grows IN the join. It's BIPHASIC - epithelial and mesenchymal tissue present with radiographic calcification
Cytogenetically: t(x;18)(p11;q11)
Metastatic to lung, bone, lymph nodes
Chemo and surgery is treatment
Pyogenic granuloma
Does not live up to its name. Capillary hemangioma that is rapidly growing with pedunculated red nodules seen on skin, gingival, or oral mucosa. My ulcerate and bleed. Pregnancy tumor
Painful soft tissue tumors
Eccrine poroma
Fine Needle aspiration
Diagnosis depends on the tissue you picked up so try to get as much as possible