Nhân 3 trường hợp bệnh sợi? (Fibromatosis)

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Tác giả: Văn Việt Thành Nguyễn, Công Quyền Trần, Tần Văn

Ngôn ngữ: Vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y học Việt Nam (Tổng hội Y học Việt Nam) 2022

Mô tả vật lý: 412-423

Bộ sưu tập: Metadata

ID: 396274

Thêm vào giỏ Liên kết toàn văn

The second case, sine 10 years ago, have had a tumor on the right pelvis which compress the ureter and the superficial artery, The thirst case, since 3 years ago, patient have had a pelvic tumor in the right side which compress the right ureter that the kidney become hydronephrosis and the tumor reccur bigger, with adominal ascite. Any case have Gardner's syndrome. On labo data, there is no IgG4, On tissue diagnosis: mimic fibromatosis (also known as fibromatosis or aggressive fibromatosis or dermoid type fibromatosis). Conclusion: They are follow up, until know. Besides the surgery, they dont's receive any drugs as anticancereux and radiotherapy.Trường hợp 1: Thiếu niên 16 tuổi, khỏe mạnh, cách 4 tháng, thấy tê chân phải và lớn dần, lại có khối u thành bụng. Chụp CT thấy khối u hố chậu bên phải, khá lớn. Khám tại BV Bình Dân, thấy khối chắc ở thành bụng bên phải. Xem hình CT cũng như vậy, Là khối u sau phúc mạc. Mổ bụng đường giữa dưới rốn, bóc tách khối u, lấy gần hết đến 90% (BS Quyền). Sau mổ, mô bướu là bệnh sợi (GS Trung). Ngày 10/12/2019, khoa Ung Bướu thấy u diễn tiến rất nhanh, nên gởi lại cho khoa TQ3. Quyết định mổ lại 14/12/2019 (GS Tần). Lần này mổ lấy khối u và nối tĩnh mạch trái - phải. Cả khối cân nặng khoảng 1 ký. Kiểm tra bàng quang, niệu đạo, không thấy xì. Sau mổ lần 2, chân bớt phù và mềm, 12 ngày sau cho bệnh về (25/12/2019), 10 ngày sau (5/01/2020) tái khám và chụp CT vùng chậu. Thấy khối u sót một ít sau xương mu và thành bụng, không triệu chứng. Cho về, kê đơn thuốc chống viêm và tái khám sau 1 tháng. Cho đi học. Tái khám sau 1 năm (12/2021), khối u lớn lên ở thành bụng phải và bẹn phải, đề nghị mổ lạinhưng người nhà chưa đồng ý. Bệnh nhân tái khám tháng 8/2022, tình trạng như cũ. Trường hợp 2: Thiến niên 15 tuổi, bị khối u ở bẹn phải. Đến khám BV Bình Dân thấy chèn ép niệu quản phải gây thận ứ nước độ 2, và chèn ép động mạch chậu ngoài phải ngày 29/9/2011. Mổ lần 1: Mổ bụng cắt bỏ bướu (GS Tần), cắm niệu quản vào bàng quang, đặt JJ (GS Chuyên). Xuất viện 28/9/2011. Mô bướu: Kết luận tăng sản sợi lành tính. (Fibromatosis) (22/09/2011: GS Trung). Lần 2: U hố chậu phải, đã mổ bụng 1 tháng, đặt JJ. Cắt bỏ gần hết khối u, dẫn lưu. Cắt ruột thừa. Cắt ruột non nhiều chỗ rách thanh mạc 28/10/2011, xuất viện 8/11/2011 (GS Tần, BS Quyền). Lần 3: Bướu tái phát rất lớn, chèn ép động mạch chậu ngoài và niệu quản phải. Mổ lại, cắt bướu, nối động mạch chậu ngoài, nối lại niệu quản - bàng quang. Bệnh nhân tái khám nhiều lần vì thận ứ nước ngày cầng nhiều, cuối cùng, đặt stent kim loại cách đây 4 tháng, thận đã ứ nước độ III (GS Vinh). Nay bệnh nhân (BN) đã đi làm, khỏe, không thấy bướu rõ. Cấp giấy hẹn chụp UIV, tái khám 18/9/2022: Tình trạng chung: Lên 5 ký, không có triệu chứng gì, khối u không sờ được, không đau bụng. Hình ảnh: khối u 50 x 42mm ở vùng chậu phải, có stent kim loại trong niệu quản, thận còn ứ nước độ III. So với hình CT trước 1 năm thì khối u nhỏ lại. Khám lần đầu đến giờ, BN không dùng bất cứ thuốc gì. Trường hợp 3: Trần Thị Mỹ L, phụ nữ trẻ, sanh 1991, bị bệnh từ tháng 8 năm 2017. U thành bụng, mổ ở BV Triều An, sinh thiết u. Mô bướu là bệnh sợi (fibrosis). 1 tháng sau, u lớn nhanh,chuyển BV Ung Bướu, không điều trị gì, BN về. Hơn 2 năm sau, BN bị suy thận cấp. Mô bướu: Là bệnh xơ (fibromatosis). Bàn luận: Là 3 bệnh, u hố chậu phải, có 1 TH kèm theo thận ứ nước độ 2, chưa có triệu chứng suy thận. Lâm sàng không có bệnh kết hợp như hội chứng Gardner. Xét nghiệm không có gì đặc biệt như IgG4. Mô bướu là bệnh xơ. Điều trị chính là mổ cắt bướu và cắt nối niệu quản và đặt stent để giải áp thận. Không có điều trị gọi là đặc hiệu như ung thư. Kết luận: Bệnh nhân được theo dõi đến nay, 2 trường hợp vẫn còn khỏe, 1 đi học và 1 đi làm, 1 phụ nữ thì bị suy thận vì mổ không lấy được khối u., Tóm tắt tiếng anh, The first case: An adolescent, 16 year old, good general state, about 6 months, paresthesia and bigger of the right thigh. He goes to BD hospital, the doctors examine him and a CT show it is a right pelvic tumor, and permit him to stay in hospital to operate. A retroperitoneal tumor which compresses the iliac vein and supercial nerve with big and hard right thigh. We operate him on 15/10/2019. Dr Quyen operates and removed about 90% tumor. After operation, The tissue diagnosis is fibromatosis. We have to sent him to oncol department to treat. On 10/12/2019, the tumor increase very fast. Prof Tan make the preop data and reoperate 14/12/2019. We remove all most tumor and anastomose the left saphene vein to the right one, because the right profound iliac vein is totally obstructed. We have to reexamine the bladder and the bilateral ureter, no wound.We continue to dissecte the left azygos vein and the right vein. We draw the left vein through the retropubic tunnel to the right vein and anastomose them end to end. We close the abdomen and the 2 anterior cutannous of 2 thighs. After the second operation, the abdomen and the right thigh decrease edema and soft. 15 days (25/12/2019), patient is out. 5/01/2020, patient come back and a CT of the pelvis. There are small tumor of the retropubis and a small tumor of the abdominal incision, but no symptoms. Prescription: An antiinflammatory drugs, and Calcium, per os. Patient come back on 12.2021, the tumor is bigger, we propose reoperation, but his mother refuse. Patient come back on 8/2022. The general state good the tumor seem bigger. The second case: An adolescent 15 year, have tumor at the right iliac region. Admitted at Binh Dan Hospital 29/9/2011 with right hydronephritis 2, and compress the superficiel iliac artery. First operation: Remove the tumor (Prof Tan), Implatation of right ureter to bladder, JJ in place (Prof Chuyen). Out of hospital 28/9/2011. Tissue diagnosis: Fibromatosis (22/09/ 2011: Prof Trung). Second operation: Recuurent tumor, remove the tumor with the terminal ileon 28/10/2011, Out of hospital 8/11/2011 (Prof Tan, Dr Quyen). Third operation: Tumor increase very fast and compress the iliac artery and right ureter. We operate to remove the tumor and anastomose the iliac artery and the ureter with stent inside. He reexamine many times because of hydronephrotis due to JJ stent, at last, a metalic stent is insecte 4 month ago, the right kidney is still good with hydronephrosis III (Prof Vinh). Now, good general state, we can't see the tumor. The next UIV is practice, patient re examine on 18.9/2022, the tumor seem smaller and thegeneral state better,with 5kg gain, no symptom. The CT mesure the tumor 2mm 50 x 4mm. The third case: Tran Thi My L, 1991, Tumor of pelvis since 8/ 2017. Operate at Trieu An hospital, only biopsy. Tissue diagnosis fibrosis. One month after, the tumor increase volume, so transfer to Oncology Hospital, no treatment specifique. Patient out of hospital. Since 2 years. a biopsy trasureteral. The tissue diagnosis: fibromatosis
Transfere to BD hospital because renal failure. The UIV show the left renal atrophy, the right renal hydronephrosis. We place JJ in the right ureter and drainage of the abdomen because big ascite and asphyxis. Re examine 26/12/2019, deliterious state. Patient requirre go back home. Discussion: So the first case, since 3 months ago, patient has the right thigh bigger than the left and right pelvis tumor.

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