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Case 33 - Discussion

Surgical Pathology Case

 

 

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Immunohistochemical staining of the solid areas

CK7, focal strong positivity

CK7

CK20, focal strong positivity

CK20

MUC1

CD68, osteoclast-like giant cells positive

Vimentin, diffuse strong positivity

Vimentin, diffuse strong positivity

 

 

Immunohistochemical staining of the cystic areas

CA19-9

CEA

CK8/18

CK19

CK7

EMA

MUC1

CD10, stroma of the cyst wall

 

IMMUNOHISTOCHEMISTRY:
Immunostains of the solid areas: beta-catenin weakly positive, CK AE1/AE3 negative, CK5/6 negative, CK8/18 negative, MUC-1 noncontributory. CK7 focally positive, CK20 focally positive, MUC1 positive, P53 focally weakly positive, CD68 the osteoclast-like giant cells strongly positive, Desmin negative, CD10 negative, MUC-1 noncontributory. Special staining PAS/H and PAS/D-LG are negative;
Immunostains of the cystic areas:: The lining epithelium of the cyst wall shows strong positivity for MUC-1, CA19-9, EMA, CK19, CK8/18 and CK7. The epithelium also shows focal positivity for CEA. The stroma of the cystic neoplasm is strongly positive for CD10.

 

 

DIAGNOSIS

 

 

SPLEEN AND PARTIAL PANCREAS, RESECTION:
- Anaplastic carcinoma of pancreas, maximal tumor dimension 8.0 cm.
   a. The anaplastic carcinoma predominantly composed of poorly differentiated spindle cells
   b. Necrosis, osteoclast-like giant cell reaction and squamoid differentiation are present.
- Mucinous cystadenocarcinoma, moderately to poorly differentiated, maximal dimension of the cyst 4.0 cm.

 

 

Reference: Z Pan, B Wang. Anaplastic Carcinoma of the Pancreas Associated with a Mucinous Cystic Adenocarcinoma: A Case Report and Review of the Literature. Journal of the Pancreas. 2007; 8(6)

 

 

 

Anaplastic Carcinoma of Pancreas

 

 

Clinical Futures

 

  • Other terms: pleomorphic carcinomas, pleomorphic giant cell carcinomas, spindle cell carcinomas, sarcomatoid carcinomas, and undifferentiated carcinoma;

  • Frequency: 2-7%;

  • More frequent in the body and tail than in the head;

  • M > F.

Gross Findings

 

  • Average size, 6 cm;

  • Cystic changes with necrosis;

Microscopic Findings

 

  • Pleomorphic large cells and spindle cells in poorly cohesive, sarcomatoid formations with desmoplastic response;

  • Tumor cells are bizarre, mononuclear or multinucleated giant cells with eosinophilic cytoplasm;

  • Cell cannibalism/tumor phagocytosis: engulfment of red blood cells or other tumor cells;

  • Osteoclast-like giant cells;

  • High mitotic rates with frequent perineural, lymphatic and blood vessel invasion;

  • Foci of glandular differentiation or squamous differentiation;

  • Tumor necrosis.

Subtypes

 

  • Spindle cell type/carcinosarcoma /sarcomatoid carcinoma: Mean survival 6 months. Predominantly spindle cells dominate with occasional giant and bizarre cells, or biphasic with epithelial/glandular and sarcomatoid components. Heterologous differentiation (cartilage, bone, striated muscle) may be present;

  • Osteoclastic giant cell type: better prognosis. contain 5 cellular components: osteoclast-like giant cells, pleomorphic large cells, histiocyte-like mononuclear cells, atypical mononuclear cells (nucleus resembles pleomorphic large cells), ductal carcinoma cells: Osteoclast-like giant cells: CD68+, keratin negative, no Kras mutation; non-neoplastic; Pleomorphic large cells (not always present): CD68-, often have Kras mutations: Histiocyte-like mononuclear cells: CD68+, keratin+, often have Kras mutations; Atypical mononuclear cells: CD68-, often have Kras mutations; Ductal carcinoma cells: CD68-, same Kras mutations as pleomorphic large cells and mononuclear cells. Molecular: osteoclast-like giant cells lack Kras mutations, but mononuclear cells have similar Kras mutations as ductal carcinoma cells

  • Pleomorphic giant cell type: mono- and multinucleated pleomorphic giant cells; pleomorphic tumor with discohesive, bizarre, multinucleated giant cells (not osteoclast-like); resembles giant cell carcinomas of lung, adrenal, liver; dense inflammatory infiltrate with emperipolesis (neutrophils in tumor cells). EMA and keratin positive

  • Round cell anaplastic type: small, round, and uniform cells, intermingled with occasional eosinophilic plump cells and giant cells.

Differential Diagnosis

 

  • Melanoma: S100+, HMB45+;

  • Pleomorphic rhabdomyosarcoma: desmin and actin +;

  • Pleomorphic sarcoma: vimentin +, cytokeratin -;

  • Metastatic carcinoma

  • Choriocarcinoma: HCG+

  • Anaplastic large cell lymphoma

  • Epithelioid sarcomas

  • Malignant fibrous histiocytoma

Immunohistochemistry Straining

 

  • CK7+, pancytokeratin+

  • Glandular component CEA+

  • Osteoclastic-like giant cell: CD68+

Election Microscopy

 

  • Microvilli and mucin.

Treatment and Prognosis

 

  • Extremely poor, 3% 3-year survival.

Cytogenetics

 

  • High frequency of K-ras mutations

Reference

 

  • AFIP

  • Int J Pancreatol. 1997 Jun;21(3):243-8

  • J Pancreas (Online) 2004; 5(6):512-515.

  • Paal E, Thompson LD, Frommelt RA, Przygodzki RM, Heffess CS. A clinicopatholgic and Immunohistochemical Study of 35 Anaplastic carcinomas of the pancreas with a review of the literature. Ann Diagn Pathol 2001; 5:129-40.

  • http://www.pathologyoutlines.com/pancreas.html

  • AJSP 1998;22:1247

  • Hum Path 2000;31:1223

  • Archives 1998;122:266

  • Archives 2002;126:1114