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To review some characteristics of the disease, the method of diagnosis and treatment attitude through a case who was treated at the Required Treatment Department, Vietnam National Cancer Hospital.
Journal of military pharmaco-medicine no5-2020 RETROPERITONEAL DESMOID FIBROMATOSIS: A CASE REPORT AND REVIEW OF LITERATURE Nguyen Tien Quang1 INTRODUCTION Desmoid fibromatosis (DF) describes a monoclonal, fibroblastic proliferation that is characterized by tendency of tumor invasion and high local recurrence, without metastatic potential DF is a rare tumor, with reported incidence of - 4% per million population DF is the most commonly found between the age of 15 and 60 years, and tends to be more common in females [1] DF is typically managed with surgical resection but a high replase However, there has not yet been an optimal therapy for this disease [4] This article aims: To review some characteristics of the disease, the method of diagnosis and treatment attitude through a case who was treated at the Required Treatment Department, Vietnam National Cancer Hospital CLINICAL REPORT - Number of medical records: 066241 - Patient: Nguyen Thi L, female, 22 years old - Occupation: Teacher - Address: Village 4, Minh Son, Do Luong, Nghe An - Hospital admission: 09/12/2019; Surgery day: 02/01/2020; Hospital discharge: 13/01/2020 - Prehistory: No history of high blood pressure, diabetes, no surgery before - Cause of hospitalization: Lower abdominal pain, numbness in left leg - Clinical features: Average physical condition, pink skin mucosa, no sign of peripheral lymph nodes, thyroid was not big Pulse: 80 beats per minute, blood pressure: 120/86 mmHg, heart rate: 18 beats per minute Height: 160 cm, weight: 42 kg Cardiovascular, respiratory examination and other organ examination revealed no injuries - Subclinical features: Blood type O (Rh+), red blood cells 4.9 T/L, leucocytes 6.3 G/L, platelets 156 G/L Blood coagulation, blood chemistry normal, HIV: Negative, HbsAg: Negative, HCV: Negative, tumor marker: CEA 0.401, CA 19.9 9.17, α FP 1.66, CA 125 18.17 - Ultrasound: The cavity behind the peritoneum on the left of lower abdominal has a hypoechoic mass of 25 mm thickness causing T ureter obstruction, hydronephrosis, lots of T inguinal node of x 11 mm size (Hypoechoic mass of 15 mm thickness in the retroperitoneal space causing the left lower ureteral obstruction, with moderate hydronephrosis Many left inguinal lymph nodes have average size of x 11 mm) Required Treatment Department, Vietnam National Cancer Hospital Corresponding author: Nguyen Tien Quang (ntienquangbvk@gmail.com) Date received: 25/3/3020 Date accepted: 15/6/2020 170 Journal of military pharmaco-medicine no5-2020 - 64 multi-slice computed tomography: Position of the left pelvic area, out of the edge of the left pelvic vascular bundles (vessels) had a mass with size of 45 x 26 mm, infused a litlle medicine after injection There were other masses nearby with similar properties located in front of the left pelvic lumbar muscle with size 53 x 26 mm touching the left pelvic but not yet invasive to the bone, enclose the left ureter in 1/3 lower segment (left pelvic ureter) causing fluid retention on the left kidney - ureter Lesions had unclear boundary with lumbar muscles of the left pelvic area with lots of inguinal lymph nodes, the largest inguinal one with size 10 x 12 mm artery invaded causing ureteral obstruction T at lower third The upper ureteral obstruction was dilated cm - Needle biopsy report 19 - 56178: Desmoid fibromatosis - Urinary crew: Release the left ureter, unable to separate the ureter from the tumor, proceed a ureter resection, tie the head of the lower ureter, release the bladder, plug the ureter into the bottom of bladder by Monosyl 4.0 layers, the inner layer is sewn with bladder mucosa, put JJ stent for T ureter Stop bleeding and suture the pelvic artery wound outside of the invasive position by Prolen 4.0, 5.0 - The patient was consulted by Urology Department and approved to place JJ stent but it was unsuccessful Then, patient underwent surgery to handle lesions - Surgery time: 180 minutes - Surgical procedures: Open abdomen by the white line at the middle and below the navel for examination: No fluid in the abdominal, smooth peritonaeum, no mass outside of two-lobe liver Nothing was abnormal in gallbladder, liver stem, stomach, duodenum, pancreas, spleen, small intestine, colorectal Behind the peritoneum and the lower abdominal, there was a rigid, immobility, fibrous tumor including large masses: The upper mass adjacent to abdominal wall muscles by x cm, the boundary was not clear and located in the T pelvic cavity, the lower mass bottom x cm besieged the inside and outside of iliac - Histopathological results: Sarcoma tumor type - Diagnosis: Retroperitoneal fibroma desmoide T lower-abdominal, slim around the pelvic vascular bundle inside and outside invaded the ureter T - Treatment: Remove the retroperitoneal tumor and the ureter, plug the ureter back into the bladder, set the JJ stent for T ureters, remove large mass above the left iliac fossa, not get the whole mass The lower mass surrounds inside and outside the pelvic vascular bundle - Place drainage of the left colon, close abdomen in double layers - Progression after surgery: The patient progressed steadily, no fever, dry incisions, sonde drained a little fluid Flatulence on 4th day - Patient was discharged from hospital on the 11th day in condition of dry incisions, no fever, transparent urine Appointment to remove JJ stent after a month was made - Pathological report after surgery: Number 20-00161, DF 171 Journal of military pharmaco-medicine no5-2020 DISSCUSSION Reviews on diagnosis and treatment Desmoid fibromatosis was found by Mankin in 1932 DF is musculoskeletal benign tumors, rarely found, accounted for 0.03% of all kinds of tumor These lesions are characterized by muscle fibroblasts proliferation with non-maglinent morphology seen clearly on a microbody but usually penetrate in situ, have a tendency of high recurrence but less metastasis; commonly combined with familial adenomatuos polyposis (FAP) caused by a germ-cell mutagens in the APC gene [1] The exact cause of DF is unclear, but it is related to history of abdominal surgery, trauma and estrogen therapy (oral contraception) FAP patient and family history which have desmoid tumor confirmed people 25% people with desmoid tumor has become a significant cause of the death rate up to 11% of FAP patient [2] Desmoid tumor in abdomen accounts for less than 10% of other types of tumor and is especially found in FAP patient The disease is associated with young women, during pregnancy as well as postpartum period and estrogen level in blood [3] Fibroids tumor usually regressively develops in menopaustrual period [1] However, in this case, patient who has not given a birth yet has no above risk factors The disease is usually asymptomatic due to the slow development of the tumor Clinical symptoms are diverse depending on tumor position, commonly abdominal pain due to obstruction syndrome, inflammatory, fever or leukocytosis Despite histologically benign and low 172 metastatic potential, DF has a tendency to invade surrounding tissues which can lead to the obstruction of structure and nearby organs This causes the progression and aggravation [4] Besides the high recurrence rate, sometimes it can be considered as fibroblastic sarcoma with low malignancy Treating these rare fibroid tumors is challenging Moreover, the invasive depth of the tumor is related to the rate of recurrence [5] Thus, surgery is very important and the main method of treatment If possible, the cut area is recommended from - cm While the role of chemicals is controversial, radiation therapy is more effective However, radiation therapy also can cause some complications [6] Disscuss about our case report Our case report was a 22-year-old woman, the age as reported in Kreuzberg’s study [5], the rate of DF was 0.73% among cases of abdominal tumor The ratio of female/male was 3/1 The disease is common in pregnant youth women or postpartum women at the age of 25 - 35 [1] Clinical manifestations in most patients was asymptomatic due to slow development of the tumor The disease manifests diversely, depending on postition and size of the tumor, clinical symptoms possibly found in abdominal dull pain, ureteral obstruction causing kidney dilation, bowel obstruction if the tumor invades into intestine and bleeding if the tumor invades into blood vessel [1] In our case, patient came for examination because of a lower abdominal pain, a vague nonspecific symptom, similarly to Destri et al’s report on patients [3] Journal of military pharmaco-medicine no5-2020 The patient was performed a CT for the lesion assessment and a needle biopsy for the histological procedure before surgery Histological result was DF, and was stained for immunohistochemistry with marker positive S - 100, positive SAM, negative Desmin, negative HMGA2, β catenin and negative CD34 The immunohistochemistry result was in accordance with DF, therefore the patient was confirmed DF before treatment - Surgery is the main treatment method, but the recurrence rate is still high Brachytherapy has the complementary role but can lead to some complication The first treatment for DF was surgical tumor resection with an area of - cm [3] depending on the tumor position A retrospective study on 189 patients treated at Anderson Cancer Center showed a status of resection surgery on microbody as prognostic factors, which were determinants of recurrence rate In our case, patient was operated to remove the tumor However, there were some blood vessel lesions at cut-off Therefore, after postoperative period is stable, the advice given is to conduct a consultation with radiation doctors for complementary radiation therapy Church J, Lynch C, Neary P A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis Dis Colon Rectum 2008; 51:897-901 CONCLUSION Through a case of a 22-year-old woman diagnosed as DF, undergoing a surgery, we draw some remarks: - Desmoid fibromatosis is a rare disease Its etiology and pathogenic mechanisms are still unclear REFERENCES Shinagare AB, Ramaiya NH, Jagannathan JP, et al A to Z of desmoid tumors AJR AM J Ronentgenol 2011; 197:W1008-W1014 Penel N, Coindre JM, Bonvalot S, et al Management of desmoid tumours: A nationwide survey of labelled reference centre networks in France Eur J Cancer 2016; 58:90-96 Penel N, Chibon F, Salas S Adult desmoid tumors: Biology, management and ongoing trials Curr Opin Oncol 2017; 29(4):268-274 Crago AM, Denton B, Salas S, et al A prognostic nomogram for prediction of recurrence in desmoid fibromatosis Ann Surg 2013; 258:347-353 Keus RB, Nout RA, Blay JY Results of a phase II pilot study of moderate dose radiotherapy for inoperable desmoid-type fibromatosis - an EORTC STBSG and ROG study (EORTC 62991-22998) Ann Oncol 2013; 24:2672-2676 173 ... about our case report Our case report was a 22-year-old woman, the age as reported in Kreuzberg’s study [5], the rate of DF was 0.73% among cases of abdominal tumor The ratio of female/male was 3/1... Shinagare AB, Ramaiya NH, Jagannathan JP, et al A to Z of desmoid tumors AJR AM J Ronentgenol 2011; 197:W1008-W1014 Penel N, Coindre JM, Bonvalot S, et al Management of desmoid tumours: A nationwide... Through a case of a 22-year-old woman diagnosed as DF, undergoing a surgery, we draw some remarks: - Desmoid fibromatosis is a rare disease Its etiology and pathogenic mechanisms are still unclear