GUT-C 11/30/2017. Debasmita Das, M.D. PGY-1 Danbury Hospital

Transcription

1 GUT-C 11/30/2017 Debasmita Das, M.D. PGY-1 Danbury Hospital

2 CLINICAL SUMMARY 8/ year old female Presented to the ED with 1 month history of general malaise, fever and weight loss PMH: Significant for a peritoneal neoplasm diagnosed 4 years prior to presentation

3 CT ABDOMEN & PELVIS A large mass in the left upper quadrant of the abdomen (13 cm x 9 cm) Inseparable from the splenic flexure of the colon and the greater curvature of the stomach Contained air bubbles compatible with necrosis

4 CT ABDOMEN AND PELVIS WITH INTRAVENOUS CONTRAST MASS Orally ingested contrast is seen to reach the colon without obstruction.there is a large mass in the left upper quadrant of the abdomen measuring 13 cm x 9 cm inseparable from the splenic flexure of the colon and the greater curvature of the stomach. There is a small amount of gas within the mass compatible with necrosis, superinfection is not excluded.. There is no evidence of free intraperitoneal air or drainable abscess detected. There is diffuse diverticulosis of the sigmoid colon without evidence of diverticulitis. ABDOMINAL WALL/PERITONEAL CAVITY: Within normal limits.

5 CT-GUIDED CORE BIOPSY - 8/22/2017 4x 40x

6 TREATMENT - 8/31/2017 Tumor debulking surgery/resection Distal gastrectomy Left and transverse colectomy Splenectomy Partial hepatectomy

7 RESECTION Peritoneal mass-attached segment of the stomach, duodenum, transverse colon, and liver. The transverse colon is opened longitudinally to reveal an area of perforation. The tumor appears to grossly protude at the site of perforation with central necrosis. The stomach is opened longitudinally to reveal a mass protruding from the wall of the stomach. The mass grossly extends through the wall of the stomach. The mass is inseparatable from the stomach wall and from the splenic flexure of the transverse colon. No gross lesion is identified in the parenchyma of the liver

8 SPLENIC FLEXURE PERFORATION STOMACH

9 TUMOR PUSHING INTO STOMACH WALL.

10 TUMOR INVADING THE COLON

11 2X TUMOR AND COLON

12 10x 40x

13 2x PERIGASTRIC MASS

14 10x 40x

15 LIVER 2x

16 LYMPH NODE 40x

17

18 CYTOKERATIN AE1/AE3 CYTOKERATIN 5/6 D2-40

19 CALRETININ WT-1 ACTIN

20 DESMIN c-kit CD 34

21 DIAGNOSIS?

22 MALIGNANT MESOTHELIOMA, SARCOMATOID/SPINDLE TYPE INVOLVING THE PERITONEUM WITH PERFORATION INTO TRANSVERSE COLON LUMEN, INVASION INTO STOMACH WALL AND FOCAL INVASION INTO LIVER CAPSULE. 2 of 4 LYMPH NODES POSITIVE FOR METASTATIC MESOTHELIOMA

23 FOLLOW-UP 10/ day h/o persistent nausea and fever CT ABDOMEN & PELVIS Extensive new liver metastases. Tumor recurrence in the left upper quadrant along the diaphragm with enlarging plaque in the right lower chest that may be due to tumor. Patient died on 10/24/2017

24 DIFFERENTIAL DIAGNOSIS OF SARCOMATOID TUMORS EPITHELIAL ORIGIN Carcinosarcoma Scirrhous/sarcomatoid hepatocellular carcinoma Sarcomatoid RCC MESENCHYMAL ORIGIN Malignant mesothelioma GIST Leiomyosarcoma Synovial sarcoma Desmoid fibromatosis Malignant solitary fibrous tumor Liposarcoma Myofibrosarcoma Fibrosarcoma Rhabdomyosarcoma Neurogenic sarcomas Endometrial stromal sarcoma Angiosarcoma

25 SARCOMATOID PERITONEAL MESOTHELIOMA

26 Am J Surg Pathol Volume 39, Number 11, November 2015

27 DEMOGRAPHICS Pure sarcomatoid peritoneal mesothelioma-exceptionally rare Median age of diagnosis: 66 years Male predominance 3.25:1 Peritoneal mesothelioma- more common in women in absence of asbestos exposure Link between asbestos exposure and peritoneal mesothelioma: less strong than in pleural mesothelioma

28 CLINICAL CHARACTERISTICS Most common presentation-abdominal symptomatology Male-abdominal pain, distension and weight loss Female-cholecystitis/cholelithiasis

29 2015 WHO CLASSIFICATION : Epithelioid : MC, best prognosis Polyclonal, oval or cuboidal cells -Tubopapillary/papillary -solid -adenomatoid -acinar -Rarer patterns -clear cell, trabeculae, small cell, rhabdoid, signet ring, pleomorphic Sarcomatoid: Rare, worst prognosis Spindle cells Desmoplastic: Bland cells within dense collagenous stroma May have Lymphohistiocytoid cells/heterologous rhabdomyosarcomatous, osteosarcomatous or chondrosarcomatous elements Biphasic Both epithelioid and sarcomatoid areas within the same tumor

30 Sarcomatoid Malignant Mesothelioma Neoplastic spindle cells in fascicles or sheets Haphazard arrangement Increased mitotic activity, necrosis, and cytologic atypia Worst prognosis Secondary growth patterns: anaplastic and giant cells; osteosarcomatous; chondrosarcomatous

31 Sarcomatoid Malignant Mesothelioma- Desmoplastic Dense collagen in storiform pattern in >50% tumor Patternless pattern of Stout Hyalinized and paucicellular whorled collagen Bland necrosis

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33 LOSS OF BAP-1 IS VERY UNCOMMON IN SARCOMATOUS AND DESMOPLASTIC MESOTHELIOMA AT ANY SITE

34 IMMUNOHISTOCHEMICAL STAINS Cytokeratins: HMW AND LMW (R/O Sarcomas) -Keratin negative sarcomatoid mesothelioma -Keratin positive sarcoma: Malignant SFT, angiosarcoma, synovial sarcoma Vimentin (Positive for fibrosarcoma) D2-40 Calretinin (Positive for fibrosarcoma)

35 IMMUNOHISTOCHEMICAL STAINS

36 DIFFERENTIAL DIAGNOSIS GIST LEIOMYOSARCOMA SYNOVIAL SARCOMA SOLITARY FIBROUS TUMOR DESMOID FIBROMATOSIS

37 GIST MAY HAVE SPINDLE CELL, EPITHELIOID OR MIXED CELL PATTERN POSITIVE STAINS: CD117, CD34, DOG1, VIMENTIN

38 LEIOMYOSARCOMA FASCICULAR GROWTH PATTERN (BUNDLES INTERSECT AT RIGHT ANGLES) TUMOR CELLS MERGE WITH BLOOD VESSEL WALLS POSITIVE STAINS: HHF35, SMA, VIMENTIN, DESMIN

39 SYNOVIAL SARCOMA MONOPHASIC-LACK EPITHELIAL CELLS BIPHASIC-SPINDLE CELLS WITH PLUMP EPITHELIAL CELLS FORMING GLANDS/CORDS POSITIVE STAINS: CYTOKERATIN 7, 8/18, 19, AE1/AE3, EMA, VIMENTIN

40 SOLITARY FIBROUS TUMOR PATTERNLESS ARCHITECTURE OF HYPO- AND HYPERCELLULAR AREAS SEPARATED BY THICK HYLANIZED COLLAGEN CRACKING ARTIFACT, STAGHORN VESSELS, PERIVASCULAR SCLEROSIS POSITIVE STAINS: CD34, CD99, BCL2, EMA, ACTIN

41 DESMOID FIBROMATOSIS CELLS ARE BIPOLAR FIBROBLASTS AND MYOFIBROBLASTS REDUCED AMPHOPHILIC CYTOPLASM THAT MERGES WITH SURROUNDING COLLAGEN, OPEN CHROMATIN, DISTINCT NUCLEOLUS POSITIVE STAINS: VIMENTIN, EMA, SMA, HHF35, NUCLEAR BETA- CATENIN, VARIABLE CD117.

42 PROGNOSIS Prognostic indicators for peritoneal MM -tumor morphology -sex (worse prognosis in males) Sarcomatoid peritoneal mesothelioma -worst prognosis -overall median survival: 5 months Younger population-increased long term survival

43 REFERENCES Pavlisko E, Roggli V. Sarcomatoid Peritoneal Mesothelioma, clinicopathologic correlation of 13 Cases. Am J Surg Pathol. 2015;39: Husain A. et al. Guidelines for pathologic diagnosis of malignant mesothelioma 2017 update of the consensus statement from the International Mesothelioma Interest Group. Arch Pathol Lab Med. doi: /arpa RA Tandon R. et al. Immunohistochemistry in peritoneal mesothelioma A Single- Center Experience of 244 Cases. Arch Pathol Lab Med. Doi: /arpa OA