A 66-year-old male with a history of AHT complaining of progressive increase in abdominal perimeter of 2 years of evolution with mild edema in the lower extremities without other accompanying symptoms Anorexia for a year and a half and progressive constipation.
To the exploration.
Aortic stenosis cardiac: systolic murmur II/VI in focus.
Isolated extrasystoles.
Pulmonary consolidation: crackles on the right base.
Globulous abdomen, tension with peripheral collateral circulation.
Mild lower extremities edemas that leave fovea to the knees
Physical examination was normal.
Paracentesis is attempted in which no liquid is obtained.
The complementary tests performed included hemoglobin: 12.3 g/dl, hematocrit: 36.7% ADE: 1.
White and platelet series were normal.
Coagulation: fibrinogen: 510 mg/dl. Normal residue.
Biochemistry: glucose 123 mg/dl. Iron 46 g/dl (65-165) urea, acid total glycase and total calcium, phosphorus, total cholesterol, HDL, triglycerides, total bilirubin, GGTfat, alkaline GPT,
Tumor markers: CEA, Ca 19.9 and alpha-Fetoprotein were normal.
Repeatedly normal tumor markers.
24-hour urine analysis was normal.
Serology for hepatitis B and C negative.
Proteinogram normal.
ECG: sinus rhythm.
Thoracic radiology: no alterations were observed.
Abdominal ultrasound: non-free fluid, multiple nodules with mass effect.
Liver, gallbladder and kidneys were normal.
Abdominal CT : numerous abdominal masses that cause displacement in the rest of the organs and are located at the peritoneum and mesentery level, the largest one measures 20 cm in anteroposterior skull diameter - 28 cm.
They have fat density and some qualifications, as the first diagnostic possibility liposarcoma.
Normal liver, spleen and kidneys
1.
Technical impossibility of performing laparoscopy due to a large number of adhesions.
FNAC: multiple punctures are performed, without being able to obtain material for the diagnosis.
Surgical intervention was performed with intraoperative diagnosis suggestive of myxoid liposarcoma.
Intraoperative macroscopic description: myxoid tumor highly suggestive of myxoid liposarcoma.
The definitive pathological diagnosis is well differentiated sclerosing liposarcoma with myxoid areas and pleomorphic areas.
Large intestine segment without definitive histological alterations.
The macroscopic description shows a mass with maximum dimensions of 25 x 22 x 14 cm, which shows a well-defined, but not encapsulated, lobulated surface.
Serial sections show that it consists entirely of a mucoid surface tissue with areas of yellow appearance and soft consistency.
Isolated necrotizing areas are observed.
Later another formation of similar characteristics is received, which has maximum dimensions of 31 x 25 x 15 cm. Likewise, a small irregular fragment of tissue 5 x 4 x 2 cm of similar characteristics to the previous two.
A segment of large intestine with a length of 4.5 cm is identified. Microscopic description shows that it consists of adipose mesenchymal proliferation with the presence of well-differentiated adipocytes of variable sizes and lipoblasts together with extensive lipoblasts.
Areas of myxoid matrix with arboriform vascular pattern are frequently observed, as well as extensive areas with the presence of markedly pleomorphic cells with a often multinucleated stellate pattern, with large and eosinophilic cytoplasm.
Areas of necrosis with polymorphonuclear leukocytes are observed among the cellular infiltrates.
The neoformation has no capsule.
The large bowel segment showed no significant histological changes or evidence of tumour inversion.
Subsequently, chemotherapy and complementary radiotherapy were performed.
Two months after the surgical intervention in the control abdominal CT, the patient presented a soft tissue mass in the left external iliac chain 7 x 3 cm in diameter, compatible with an adenopatic bladder that caused right lateral displacement of the bladder.
Six months after the first operation, a new left retroperitoneal tumor extending to the left sacroiliac joint was operated on.
Three quarters of the tumor were removed leaving tumor on the left iliac and sacroiliac veins. The anatomopathological result of the retroperitoneal nodules was the macroscopic description of: irregularly nodular homogeneous fragments 18 x 13 cm showed a brown appearance.
Microscopically, a biphasic mesenchymal neoplasia with predominance of areas of high-grade non-lipogenic sarcoma appears, of which emerge in continuity, areas of atypical tumor with sclerotic prominent pattern.
Areas of non-lipocytic sarcomas present histological images similar to those of malignant fibrotiocytoma, with zones of more than 5 mitoses x 10 high-power fields, occasionally due to the presence of dysplasias.
fusiform cellularity with numerous pleomorphic, multinucleated and gigantonucleated cells.
In other areas the cells have spindle characteristics.
No differentiation to lipoblasts has been observed between the mesenchymal cells described.
The other component of biphasic neoplasia shows numerous adipocyte cells with a slightly fibrous stroma with component of greater nuclear polymorphism.
Immunoexpression of p53 is 5-10% in nuclei, with a higher proportion in areas of non-lipogenic sarcoma.
It is interpreted as a sign of tumor progression.
A lymph node may be seen in one of the peripheral areas of the tumor, as infiltrated by the tumor.
The described mesenchymal neoplasm is accompanied by a moderate inflammatory component, intermingled with mesenchymal cells, this constitutes a high-grade neoplasm compatible with dedifferentiated liposarcoma.
Palliative radiotherapy is performed with good tolerance to it and consecutive controls of thoracic and abdominal CT without significant radiological changes until at 3 years appears in the middle lobe of the right lung an anteroposterior, hypodense diameter 2.5 cm, 5 cm or 5 cm lesion.
Abdominal CT does not show pathological abdominal or pelvic lymphadenopathies, as well as a soft tissue tumor with poorly defined borders in the internal zone of the iliac bone left muscle that does not displace the iliac vessels.
These findings suggest recurrence or metastasis of the intervened retroperitoneal liposarcoma.
