병리과 4학년 PK 6조 33번 박경선 3학년 PK 24조 10번 고영주 Case Presentation 병리과 4학년 PK 6조 33번 박경선 3학년 PK 24조 10번 고영주

Identifying Data Name : 김 O O Sex : Female Age : 28

Chief Complaint Lower abdominal painful mass for 1 month

Present Illness 내원 30일전 sudden onset painful mass at lower abdominal area에 develop 되었으나 observation하였고 내원 10일전 intermittent pain으로 HB visit하여 시행한 CT상 intraperitoneal mass detect되어 biopsy 시행하였고 fibrous tissue 소견 보여 수술 위해 입원

Past Medical History pulmonary tuberculosis (-) type 2 DM (-) hypertension (-) Previous illness (-) polyposis hx(-) trauma hx(-) Previous abdominal surgery hx(-) 불임으로 본원 OBGY 외래: estrogen exposure(+)

Personal History Alcohol (-) Smoking (-) Allergy (-) Occupation (-)

Family History pulmonary tuberculosis (-) type 2 DM (-) hypertension (-) Familial adenomatous polyposis(-)

Physical Examination Vital sign : stable General : alert HEENT     : not pale conjunctiva CHEST     : CBS s crackle Lung        : Clear breathing sound s crackle Heart       : Regular heart beat s murmur Abdomen  : soft & flat normoactive bowel sound lower umbilicus area  5*5cm sized mass(+) & Td/RT(+/-) Extremities : no pitting edema

Abdominal CT ( 07.5.30 ) Supravesical abdominal cavity내에 약 6 x 4 x 6.4 cm size의 enhancing mass mass의 margin는irregular하며 내부에 small fat componant가 관찰됨. 인접한 bowel loop에 wall thickening(-) 복강 내에 의의 있게 커진 lymph node(-) Liver. GB, biliary trees, pancreas, spleen, kidneys gross abnormality(-)

CT-guided bx(07.6.1) Abdominal wall, lower portion, needle biopsy: Fibrous tissue

Impression R/O desmoid tumor R/O GIST R/O leomyoma R/O leomyosarcoma R/O neurogenic tumor

Operation (07.6.15) Procedure Segmental resection of small bowel          Excision of mesnechymal tumor         Incidental appendectomy Finding 10*10cm size의 well demarcated, hard nature tissue 가 Treiz lig.하방 120cm의 mesentery에 location되어 있으며 주변의 small bowel과 severe adhesion(+)  but 다른 peritoneal tissue와는 adhesion (-) Normal appendix but minimal inflammatory change(+) Procedure : Rt Hemicolectomy              Close suction drainage Indications : Colonoscopy c Bx ; A-C ca, adenoca               CT ; T3N0 Finding and Technique : OP Findings ; Rt colon에 6cm 크기의 bulky mass 관찰됨 OP Techniques ; GIA #100 2개 

Gross Finding A poorly demarcated rubbery to firm and infiltrative mass (5.5x4.5x4.5cm) is present in the intestinal mesentery. The cut surface of the mass is whitish tan and trabeculated. Necrosis(-) hemorrhage(-) GROSS: The gross specimen is a right hemicolectomy composed of a part of ascending colon, cecum and terminal ileum. The large intestine measures ( 22.0 )cm in length and ( 4.8 ) cm in diameter. The terminal ileum measures ( 4.5 )cm in length and (3.5 )cm in diameter. The serosal surface of cecum is irregularly thickened in the tumor portion. On opening the specimen, there is a huge, ulceroinfiltrating tumor with elevated margin, ( 4.5 )cm from the proximal resection margin. The tumor mass measures (5.6x4.5)cm in size, respectively. The cut surface of the tumor shows pale tan to white, firm and friable tissue, extending to the serosal layer. The remaining mucosa of the colon shows unremarkable. Total ( ) regional lymph nodes are found in the pericolic adipose tissue. An appendix ( 7.0 )cm in length and ( 0.6 )cm in maximum diameter, is attached to the cecum. Gross photo present. Blocks T1-4, tumor mass and surrounding tissue x4 P, proximal resection margin x 1 D, distal resection margin x 1 LI, large intestinal mucosa x1 X, appendix x 1 LN1-5, regional lymph nodes x 5

Microscopic Finding Composed of broad, sweeping fascicles of monotonous spindle cells Bland nuclear feature and finely collagenous stroma cellularity가 크게 높지 않고 nest 형성(-) C-kit(-) CD34(-) SMA(-) desmin(-) S100(-)

Pathologic Diagnosis Mesenteric fibromatosis

Mesenteric fibromatosis Disease Review Mesenteric fibromatosis

Introduction m/c primary tumor of the mesentery Location Most-mesentery of the small bowel Some-originate from ileocolic mesentery, gastrocolic ligament, omentum, retro-peritoneum

Etiology Sporadic (m/c) prior surgical intervention(abdominal) Trauma With familial adenomatous polyposis patients with elevated serum estrogens during pregnancy postpartum male alcoholic with bilateral gynecomastia.

Date on age and gender vary Symptom most- asymptomatic abdominal mass some have mild abdominal pain 드물게, gastrointestinal bleeding or bowel perforation Date on age and gender vary Kim et al: Male > Female Yannopoulos: Male = Female Burke et al: Male < Female

Many have an initial phase of rapid growth Complications: Size: large, 10 cm or more Many have an initial phase of rapid growth Complications: compression of the ureter ureteral fistula compression of the small or large intestines intestinal perforation에 의한 complication

Pathologic finding Grossly: fairly well circumscribed Microscopically: typically infiltration into the surrounding soft tissues, including the bowel wall spindle-shaped or stellate cells deposited in a densely collagenous stroma. variable cellularity stroma shows marked myxoid change.

Gross finding

Microscopic finding

Microscopic finding

Differential Diagnosis sclerosing mesenteritis mesenteric panniculitis mesenteric lipodystrophy => 1. characterized by fibrosis 2. prominent chronic inflammatory infiltrate that abuts 3. but does not penetrate, bowel wall

Differential Diagnosis Inflammatory fibrosarcoma of the mesentery and retroperitoneum => 1. more cellular, cytologic atypia 2. less fibrotic 3. more inflamed

Differential Diagnosis Idiopathic retroperitoneal fibrosis associated with methysergide administration inflammatory abdominal aortic aneurysm =>more densely hyalinized, inflamed

Recurrence propensity for local recurrence difference in recurrence rates btw. patients with Gardner syndrome(90%) -some patients died of tumor without Gardner syndrome(12%) -no patients died of tumor

Mesenteric Fibromatosis in Gardner Syndrome Mesenteric or retroperitoneal fibromatosis onset 1~2 years after excision of the diseased portion of the intestinal tract most tumors grow slowly Histologically indistinguishable polyposis-related cases, sporadic cases by morphology alone

Mesenteric Fibromatosis in Gardner Syndrome Recurrence common higher rate of recurrence in polyposis-related fibromatosis than in sporadic cases Treatment Endocrine therapy (tamoxifen, prednisolone) noncytotoxic drug therapy (sulindac, indomethacin) chemotherapy

Mesenteric Fibromatosis in Gardner Syndrome Complete excision: often difficult => necessitates removal of a sizable segment of the intestine together with the fibrous growth Rapidly growing tumor excision => attempted to prevent complications caused by the presence of a massive, expanding intraabdominal growth

Treatment of Mesenteric Fibromatosis similar to extraabdominal fibromatosis excision is often difficult: irregular growth pattern and interstinal attachment of the tumor. Other modes of therapy antiestrogenic agents Steroids cytotoxic chemotherapy postoperative irradiation

Quiz를 풀어봅시다…

Quiz 1 28세 여자환자가 sudden onset severe abdominal pain을 주소로 내원하였다. 다음은 내원 당시 Abdominal CT사진이다.

7일후 laparotomy결과 large mass가mesentery와 bowel wall내에 infiltration되어 있었다 7일후 laparotomy결과 large mass가mesentery와 bowel wall내에 infiltration되어 있었다. Resection했으며 Bx결과는 다음과 같다

Immunohistochemistry 결과는 다음과 같다 B-catenin(+) c-kit(-), CD34(-) S100(-), SMA(-) 가능한 진단은? ① GIST ② leiomyoma ③ neurogenic tumor ④ mesenteric fibromatosis ⑤ lymphoma

Quiz 2 가. 대부분 소장의 장간막에서 생긴다. 나. 대개는 abdominal surgery후 생긴다. Mesenteric fibromatosis의 내용 중 틀린 것은? 가. 대부분 소장의 장간막에서 생긴다. 나. 대개는 abdominal surgery후 생긴다. 다. Tumor 크기는 10cm정도로 큰 편이고, 소장or 대장을 압박하기도 한다. 라. 주변 장기와 경계가 분명하여 tumor만 절제 하기 쉽다. ① 가,나,다 ② 가,다 ③ 나,라 ④ 라 ⑤ 가, 나, 다, 라, 마

Quiz…kidney 가. 조직검사상 severe proliferation & sclerosis 22세 남자가 군신검상 시행한 요검사에서 현미경적 혈뇨가 있어 내원하였다. USG, IVP는 정상소견이었으며 Renal bx.소견은 아래와 같다. 이 환자의 나쁜 예후와 관련 있는 인자는? 가. 조직검사상 severe proliferation & sclerosis 나. Gross hematuria 다. Serum creatinine 상승 라. 혈청 보체 증가 ① 가,나,다 ② 가,다 ③ 나,라 ④ 라 ⑤ 가, 나, 다, 라, 마

Ig A C3 Lamda Kappa