Case presentation. Awatif Al-Nafussi

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1 Case presentation Awatif Al-Nafussi

2 Case History 49 year old DVT & small PE June 08, Pelvic mass Ca Laparotomy-TAHBSO, drainage of ascites

3 Ovarian carcinoma Clinical diagnosis

4 Multiple specimens 1 uterus, cervix, both tubes & ovaries 2. right adnexal tumour 3. omentum 4,5,6 nodules from sigmoid, rectum and peritoneum 7 appendix 8 biopsy from small bowel 9 ascitic fluid

5 Gross finding Both ovaries appear normal, but 9 mm nodule in left ovary Right adnexal tumour-lobulated, solid, partly necrotic-220 mm Omental nodules 15 & 9 mm respectively All other nodules-solid tumour

6 Left ovary Right ovary

7

8 Right adnexal tumour

9

10 Omental nodule Nodule from small bowel

11

12

13 Ascitic fluid

14 Malignant epithelial/epithelioid tumour with tubular pattern What is your diagnosis? 1. Ovarian carcinoma 2. Epithelioid mesothelioma 3. Intra-abdominal small round cell desmoplastic tumour-pseudo glandular pattern 4. Epithelioid leiomyosarcoma

15 Special stains AB/PAS Immunostains

16 AB/PAS

17 CAM5.2 VIM CK7

18 1. Ovarian carcinoma 2. Epithelioid mesothelioma 3. Intra-abdominal small round cell desmoplastic tumour-pseudo glandular pattern

19 DES

20 Possible diagnosis 1. Epithelioid mesothelioma 2. Intra-abdominal small round cell desmoplastic tumour-pseudo glandular pattern

21 CK5/6 CALR HBME1 CA125

22 CD99 WT1 ER CKIT

23 CD34 SMA NSE

24 Very unusual Immunostains Immunoprofile favour intra-abdominal desmoplastic tumour But H & E favoured mesothelioma

25 Cytogenetic Negative for Small cell intra-abdominal desmoplastic tumour Sent for various second opinion-all favoured mesothelioma

26 Epithelioid mesothelioma can grow with 4 different patterns: tubular, papillary and diffuse or combination of all three

27 Malignant peritoneal mesothelioma a rare aggressive tumour of the peritoneum, regarded as a universally fatal disease (median survival 6-12 months Only 20% to 33% of all mesotheliomas arise from the peritoneum (visceral or parietal) asbestos, is the principal risk factor It can occur in any age group (50- to 69-year)

28 Symptoms Most common Abdominal pain (35%), Abdominal swelling (31%), Anorexia, marked weight loss, and ascites. Less frequently night sweats and hypercoagulability.

29 Peritoneal mesothelioma usually remains confined to the peritoneal cavity for most of its natural history. Typical growth pattern of peritoneal mesothelioma is locally expansive masses. Haematogenous or lymphatic metastasis is unusual

30 Markers for Mesothelioma Positive EMA Calretinin Thrombomodulin CD44H HBME-1 WT1

31 Negative CEA TTF1 Ber-EP4, CD34

32 Variant s of mesothelioma Epithelioid Sarcomatoid Biphasic Desmoplastic Lymphohistiocytoid Decidoid

33 Desmoplastic mesothelioma

34 Lymphohistiocytoid mesothelioma Very rare variant Large discohesive histiocytic-like cells admixed with intense lymphoplasmcytic cells

35

36

37

38 PAN-CK

39 CK 7 CD45 EMA CALRE

40 Deciduoid mesothelioma Large, polygonal or ovoid cells with well-defined cell borders, dense eosinophilic cytoplasm, and single or multiple nuclei frequent loss of cell cohesion, marked nuclear atypia, and high mitotic activity (>5 per 10 HPF);

41 Deciduoid mesothelioma initially considered to occur exclusively in the peritoneum of young women who had no history of asbestos exposure and to be characterized by an aggressive clinical course, but it was later demonstrated that this tumour could also occur in the pleura of older men and women who had been exposed to asbestos.

42 Other mesothelial lesions

43 Papillary mesothelioma Mesothelial hyperplasia Multicystic mesothelioma

44 69 year old History of exposure to asbsetos?ovarian carcinoma?primary peritoneal carcinoma? Mesothelioma Immuno:CK5/6, calretinin, MNF116 and Ca125 all strongly positive Thrombomodulin -weak positively

45

46

47

48 Immunostains CK5/6, calretinin, MNF116 and Ca125 all strongly positive Thrombomodulin -weak positively

49 Well-differentiated papillary mesothelioma Usually in women of reproductive age No history of asbestos exposure Indolent course and good prognosis Incidental or presents as ovarian mass Recurrence is rare

50 Florid mesothelial hyperplasia Occurs in pelvic or abdominal peritoneum Often seen in association with large ovarian tumours Predisposing factors: Primary or secondary pelvic tumours Ascites Endometriois

51 80 year old-reviewed with large left sided mucinous ovarian tumour, plaque on the surface

52

53 Surface nodule

54

55 Referral diagnosis?separate serous tumour?clear cell carcinoma

56 Review diagnosis? Florid mesothelial reaction

57 Desmin CK7 Fallopian CK5/6 tube Calretinin

58 Ber-EP4 ca125 CEA ER

59 Benign Multicystic mesothelioma Young women Often associated with previous surgery, endometriosis or inflammation Often multifocal Formation of multiple, thin-walled, multilocular cysts that frequently produce large, intraabdominal masses.

60 39 year old woman History of endometriosis Cyst found in pouch of Douglas Grossly mutltilocular cyst

61

62 references 1. Paul H Sugarbaker, Yair I.Z Acherman, Santiago Gonzalez-Moreno, Gloria Ortega-Perez, O.Anthony Stuart, Pierre Marchettini, Dal Yoo Diagnosis and treatment of peritoneal mesothelioma: The Washington Cancer Institute experience. Seminars in OncologyVolume 29, Issue 1, February 2002, Pages A. Malpica, S. Sant'Ambrogio, M. T. Deavers, and E. G. Silva. Well-differentiated papillary mesothelioma of the female peritoneum: a clinicopathologic study of 26 cases. Am.J.Surg.Pathol. 36 (1): , Wolanski KD, Whitaker D, Shilkin KB,Henderson DW. The use of epithelial membrane antigen and silver-stained nucleolar organizer regions testing in the differential diagnosis of mesothelioma from benign reactive mesothelioses.cancer 1998;82: Saad RS, Cho P, Liu YL, Silverman JF. The value of epithelial membrane antigen expression in separating benign mesothelial proliferation from malignant mesothelioma: a comparative study. Diagn Cytopath 2005:32(3): Ordóñez, Nelson G MD Value of Immunohistochemistry in Distinguishing Peritoneal Mesothelioma From Serous Carcinoma of the Ovary and Peritoneum: A Review and Update.Advances in Anatomic Pathology:January Volume 13 - Issue 1 - pp Clement, Philip B. M.D.; Young, Robert H. M.D.; Scully, Robert E. M.D. Malignant Mesotheliomas Presenting as Ovarian Masses: A Report of Nine Cases, Including Two Primary Ovarian MesotheliomasAmerican Journal of Surgical Pathology:September Volume 20 - Issue 9 - pp F. Tresserra, M. Castella, G. Fabra, Martinez M. Angeles, M. Dominguez, C. Fernandez-Cid, M. D. Amalrich, and C. Ramos. Lymphohistiocytoid mesothelioma of the pleura: A case report with cytological findings. Diagn.Cytopathol., N. G. Ordonez. Deciduoid mesothelioma: report of 21 cases with review of the literature. Mod.Pathol., H. Mani and M. J. Merino. Mesothelial neoplasms presenting as, and mimicking, ovarian cancer. Int.J.Gynecol.Pathol. 29 (6): , D. Dellaportas, E. Kairi-Vassilatou, P. Lykoudis, P. Mavrigiannaki, S. Mellou, C. K. Kleanthis, and A. Kondi-Pafiti. Peritoneal mesotheliomas mimicking adnexal tumors. Clinicopathological characteristics of four cases and a short literature review. Eur.J.Gynaecol.Oncol. 33 (1): , Izhar Bagwan, Nirmala A. Jambhekar, Ratnaprabha K. Ghodke, Uma Bansal.Florid Tubulopapillary Mesothelial Hyperplasia: A Lesion Mistaken for Carcinoma a Case Report. journal of Gynecologic Surgery. Mar 2005, Vol. 21, No. 1: N. E. Terry and C. L. Fowler. Benign cystic mesothelioma in a child. J.Pediatr.Surg. 44 (5):e9-11, 2009.

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