Leiomyosarcoma differential diagnosis

• Abnormal vaginal bleeding (Postmenopausal or intermenstrual bleeding or bleeding from uterus or vagina)
• Pressure or pain pelvic or abdomen
• Vaginal discharge
• Change in bladder or bowel habits
• Fatigue
• Fever
• Malaise
• Weight loss

• Lump protruding from vagina

• Mass in abdomen or pelvic

• Tenderness on palpating pelvic or abdomen

• Lymphadenopathy

• ↓ RBC or Hemoglobin may be seen on CBC.
• In uterine leiomyosarcomas (LMSs) p16 is overexpressed compared with leiomyoma.

• Deep myometrial invasion

• Massive uterine enlargement
• Irregular central zones of low attenuation,
• Extensive necrosis and hemorrhage

• Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma.

• Gross histology:
• Large, solitary, poorly circumscribed masses (average 10 cm)
• Typically display a fleshy variegated cut surface with areas of hemorrhage or necrosis

• Histology and biopsy

• Women with sarcoma vs women with fibroids, tend to be older
• More likely to be postmenopausal
• More likely to have a history of another nonuterine malignancy
• Women with sarcoma were more likely to have masses that are subserosal
• Solitary rather than multiple uterine mass

• Vaginal bleeding

• Pelvic pain
• Urinary retention
• Constipation
• Infertility
• Bowel obstruction
• Vaginal discharge

• Large,irregular pelvic mass

• CBC suggestive of anemia
• Bcl-2 expression is more frequent and strong marker in leiomyomas compared with leiomyosarcoma.

• Enlarged uterus with multiple whorled mass lesions, largest of size 8.6×7.1 cm, not extending into the abdomen

• An enlarged uterus and a deformed uterine contour are the most common CT findings of leiomyomas
• Leiomyomas usually have a uniformly solid consistency

T2-weighted MRI:

• Hypointense masses
• Homogeneous

enhancement

• Red degeneration

• Abscence of cytologic atypia

• Mild nuclear atypia, up to 10-20 mitosis

• No tumor cell necrosis

• Biopsy and histology

• Abdominal pain
• Vaginal bleeding

• Pelvic pain
• Urinary retention
• Constipation
• Infertility
• Bowel obstruction
• Vaginal discharge

• Large,irregular pelvic mass

• ↓ RBC or Hemoglobin on CBC

• Irregularly enlarged uterus, usually hypoechoic but can be isoechoic and even hyperechoic as well.

• Enlarged uterus with irregular border

• Hypointense masses

• Zones of recent hemorrhage within nodules of hypercellular smooth muscle.

• Coagulative necrosis (red degeneration)

Mitotic figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas

On gross examination:

• Features of multiple hemorrhagic area
• Necrosis
• Cyst formation
• Softening, or color different than the usual leiomyoma

• Biopsy specimen and histology

• Palpable abdominal masses
• Abnormal vaginal bleeding
• Flank pain (kidneys)
• Dull abdominal pain in right upper quadrant (liver)

• Lower abdominal pain with a palpable mass

• PECs typically stain for melanocytic markers (HMB-45, HMSA-1, Melan A (Mart 1), micro-ophthalmia transcription factor (Mitf), myogenic markers (actin), and less commonly desmin

• Granulomatous ovoid uterine tumor with a rich vascular network

• Mean tumor diameter of 5 cm, well-defined margins, hypodense mass

• Low and high signal intensity on T1- and T2-weighted MRI
• Strong, heterogeneous enhancement

• Perivascular location
• Epithelioid, elongated and spindle shaped, or vacuolated (adipocyte-like).
• Clear to granular, lightly eosinophilic cytoplasm
• Nuclear atypia

• Biopsy

• Association with lymphangiomyomatosis and tuberous sclerosis

• Painful, enlarging soft tissue masses

• Long bone fractures
• Arteriovenous shunting and subsequent high-output cardiac failure symptoms such as dyspnea on exertion.

• Peritoneal bleeding in some cases
• Painful, enlarging soft tissue masses

• Strongly positive for vimentin
• Positive for cytokeratin
• CD31, CD34

• Nonspecific

• Bulky or normal size uterus

• Intratumoral blood flow on Doppler

• Enhanced huge enhanced round heterogenous solid mass with internal multifocal cysts on CT scan

• Focal areas of high signal intensity, known as the “cauliflower-like appearance” on gadolinium-enhanced MRI

• Nodules, and trabeculae of infiltrative epithelioid to spindled cells
• Eosinophilic cytoplasm.

• Biopsy and histology

• Epithelioid angiosarcoma has a male predilection
• Most often arises in the deep soft tissues (usually intramuscular) of the extremities, but a variety of primary sites, including the thyroid gland can be involved.
• Early nodal and solid organ metastasis, especially to the lungs, bone, soft tissue, and skin.

• Symptoms varies depending on tumor location.
• Symptoms related to uterine leiomyoma such as abdominal pain, abnormal uterine bleeding, and abdominal distension
• Dyspnea, lower extremity edema in case of cardiac involvement

• Jugular venous distension
• Lower extremity edema
• Abdominal mass

• No specific lab finding

• Vascularized thrombi within the pelvic veins and inferior vena cava

• Irregular mass exhibiting heterogeneous enhancement with extension into adjacent veins

• Leiomyomatous lesion or a distorted uterus with projections into vasculature.

• Benign, well-differentiated tumor with smooth muscle growing within veins as worm-like projections.

• Biopsy and Histology

• Echocardiography may show hyperechoic elongated mobile mass extending from the IVC and an irregular lesion in the right atrium with protrusion into the right ventricle