NORTHWESTERN UNIVERSITYÕS FEINBERG SCHOOL OF MEDICINE

ILLINOIS REGISTRY OF PATHOLOGY

OCTOBER 27, 2003

 

Case #1 Ð David M. Weinrach, MD

 

History:  A 40 year-old female with a history of symptomatic uterine leiomyomata and ascites was found to have a pelvic mass on computed tomographic scan.  She underwent a total abdominal hysterectomy and bilateral salpingoophorectomy which revealed a 15 cm. pedunculated, subserosal, uterine mass.

 

DDX:  symplastic leiomyoma, leiomyosarcoma

 

Final DX:  symplastic leiomyoma.   Comment:  the mitotic index is 2/50 hpf.  Because 2 atypical mitoses were identified, the biological behavior can not be predicted.

 

References:

Bell SW, Kempson RL, Hendrickson MR.  Problematic uterine smooth muscle neoplasms.  A clinicopathologic study of 213 cases.  Am J Surg Pathol 1994; 18: 535-558

 

Perrone T, Dehner LP.  Prognostically favorable Òmitotically activeÓ smooth-muscle tumors of the uterus:  a clinicopathologic study of 10 cases.  American Journal of Surgical Pathology  1988; 12: 1-8.

 

Downes KA, Hart WR.  Bizarre leiomyomas of the uterus:  a comprehensive pathologic study of 24 cases with long term follow-up.  American Journal of Surgical Pathology 1997; 21: 1261-1270

 

Case #2ÑDr. Kim Wang, M.D.

 

Clinical history:

The patient is a 66 year old male with a 1 year history of a 10 cm left anterior thigh mass.  The mass was initially <3 cm in size and soft to palpation.  A few months prior to presentation the mass became larger, firmer, and more painful.  Radical resection of the tumor was performed.

 

Diagnosis:

Atypical / malignant ossifying fibromyxoid tumor of soft parts

 

Differential Diagnosis:

o             Benign

n              Myositis ossificans

n              Myoepithelioma

p             Parachordoma

n              Epithelioid peripheral nerve sheath tumor

 

o             Malignant

n              Extraskeletal osteosarcoma

n              Extraskeletal myxoid chondrosarcoma

 

 

Discussion:

From:  Folpe A, Weiss SW.  Ossifying fibromyxoid tumor of soft parts.  AJSP 27 (4): 421-431, 2003.

 

 

Diagnostic criteria

# of cases

Local recurrences

Metastases

Typical OFMT

Low nuclear grade and low cellularity and mitotic rate <2/50 HPF

25

3 (12%)

1 (4%)

Atypical OFMT

Tumors deviating from typical OFMT but not meeting criteria for malignancy

16

2 (13%)

1 (6%)

Malignant OFMT

High nuclear grade or high cellularity and mitotic activity of greater than 2/50 HPF

10

6 (60%)

6 (60%)

 

 

 

Antigen

Overall positive (%)

Folpe                       Enzinger                Schofield

S-100

33 of 55 (60%)

34 of 46 (74%)

10 of 12 (83%)

Desmin

  5 of 39 (13%)

 

  7 of 10 (70%)

Pan-cytokeratin

  5 of 48 (10%)

  1 of  9  (11%)

 

SMA

  3 of 44 (7%)

 

  4  of  8 (50%)

Collagen type II

  1 of 25 (4%)

 

 

 

References:

Enzinger FM, Weiss SW, Liang CY.  Ossifying fibromyxoid tumor of soft parts:  a clinicopathological analysis of 59 cases.  AJSP 13(10): 817-827, 1989.

 

Folpe A, Weiss SW.  Ossifying fibromyxoid tumor of soft parts.  AJSP 27 (4): 421-431, 2003.

 

Donner LR.  Ossifying fibromyxoid tumor of soft parts:  evidence supporting Schwann cell origin.  Human Pathology 23(2):  200-202, 1992. 

 

Kilpatrick SE, Ward WG, Moxes M, Miettinen M, Fukunaga M, Fletcher CDM.  Atypical and malignant variants of ossifying fibromyxoid tumor:  clinicopathologic analysis of six cases.  AJSP 19(9) 1039-1046, 1995.

 

Schofield JB, Krausz T, Stamp GWH, Fletcher CDM, Fisher C.  Ossifying fibromyxoid tumor of soft parts:  immunohistochemical and ultrastructural analysis.  Histopathology 1993, 22, 101-112.

 

Case #3 - Brian Adley, MD

 

History:  The patient is a  62 year old female with a progressively enlarging mass of the left labia majora.  Past medical history includes a remote history of uterine fibroids s/p TAH.

 

Gross:

n              4.5 X 3.0 X 1.5 cm well-circumscribed subcutaneous nodule

n              Tan-pink, rubbery cut surface, not connected to the overlying skin

n              No necrosis or hemorrhage

 

Histology:

n              Uniformly cellular lesion composed of  cytologically bland cells with oval to fusiform nuclei and scant lightly eosinophilic cytoplasm set in an open collagenous matrix

n              Focal fascicular growth pattern

n              Prominent vascular component consisting of small to medium-sized vessels with uniform distribution

n              Some vessels with prominent hyalinization and perivascular chronic inflammation

 

Differential Diagnosis:

n              Cellular Angiofibroma

n              Angiomyofibroblastoma

n              Aggressive Angiomyxoma

n              Cellular Fibroepithelial Polyp

n              Hemangiopericytoma/SFT

 

Immunohistochemical Profile:

n              Vimentin, ER positive

n              Desmin, CD34, SMA negative

 

Diagnosis: Cellular Angiofibroma

 

Reference:

Nucci MR,  Fletcher CDM. Vulvovaginal soft tissue tumors: update and review.  Histopathology 2000, 36, 97-108.

Nucci MR, Granter SR, Fletcher CDM.  Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma.  AJSP 21:636-644.

Laskin WB, Fetsch JF, Mostofi FK.  Angiomyofibroblastomalike tumor of the male genital tract.  AJSP 22:6-16.

 

Case # 4 - Rohit Gupta, MD

 

History: A 74 year old female initially presented to NMH with chest and back pain for 3 weeks. Imaging studies revealed a posterior mediastinal mass arising from the distal esophagus.

Gross:  Surgical pathology received a 12.5x 10x 6.5 cm, 332 g well circumscribed mass.  Sectioning revealed a fleshy, lobulated cut surface with areas of hemorrhage and necrosis.

Histology: The tumor consisted predominantly of uniform spindle cells in fascicles and included epitheloid areas.  Focally, the tumor was highly cellular with a high mitotic rate.  Areas of tumoral necrosis were present.

Differential Diagnosis: Leiomyosarcoma and Malignant Gastrointestinal Stromal Tumor (GIST)

Immunohistochemistry:  C-kit and CD34 positive, smooth muscle Actin and Desmin negative

Our Diagnosis: Malignant Gastrointestinal Stromal Tumor

Comments

-Incidence of GIST in USA is rising.

-GISTs are thought to arise from the interstitial cells of Cajal or their precursor cells.

-GIST may occur anywhere in GI tract [stomach (60%), small bowel (20-30%), colon (5%), esophagus (2%), and rarely involve appendix and omentum].

      -Associated with CarneyÕs Syndrome and von Recklinghausen disease (NF1).

      -GISTs have a variable malignant potential that is difficult to predict; size and mitosis      are the most important indicators

      -Metastases may occur late, usual sites include liver, peritoneum and lungs.

      -Malignant GIST have poor prognosis (median survival 10-21 months).

      -Architectural patterns: spindle cell type (70%), epitheloid type (20%) and mixed

      -Different locations may show variable immunoreactivity for CD34.

      -KIT expression should be present with rare exceptions in GIST.

      -CD34 (60-70%), SMA (30-40%), S-100 (5%), and Desmin (rare) are also useful in  establishing a diagnosis of GIST

     -NIH consensus conference issued criteria for estimating malignant potential of GIST into very low risk, low risk, intermediate, and high risk categories.

     -Important size cut-offs are <2 cm, 2-5 cm, 5-10 cm and >10 cm

     -Important mitotic counts are <5/50 hpf, 5-10 hpf, >10 hpf

References:

Dei Tos, AP The reappraisal of gastrointestinal stromal tumors:from Stout to the KIT revolution. Virchows Arch: 2003; 442:421-428

Fletcher, CDM et al Diagnosis of Gastrointestinal Stromal Tumors: A Consensus Approach . Int J Surg Pathol: 2003:10(2):81-89

Goldblum, JR Gastrointestinal Stromal Tumors: A Review of Characteristic Morphologic, Immunohistochemical, and Molecular Genetic Features. Am J Clin Path: 2002;117(suppl 1):S49-61

Miettinen, M et al Pathology and Diagnostic criteria of gastrointestinal stromal tumors (GISTs): a review. Euro J of Cancer: 2002; 38 (suppl 5):S39-51

Miettinen M  Esophageal Stromal Tumors. Am J Surg Path: 2000; 24(2):211-222

Rosai, J. GIST: An Update. Int J Surg Pathol: 2003:11(3): 177-186.

 

Case #5 - Maria Luisa C. Policarpio-Nicolas, M.D.

 

History:  59 year old female with a right breast mass.  Mammography revealed dense breast parenchyma with benign appearing calcifications and a 7 mm hyperechoic lower inner quadrant nodule was identified on sonography.  Past screening mammography was negative.  She had a contralateral intraductal papilloma.  She was otherwise in good health.  A biopsy was performed (kodachrome slide in your packet) for which a diagnosis of carcinoma was rendered.   She eventually had a lumpectomy (glass slide in your packet).

 

Differential diagnosis:

1.              Microglandular Adenosis (MGA)

2.              Atypical Microglandular adenosis (AMGA)

3.              Adenoid Cystic Carcinoma (solid, tubular, cribriform)

4.              Tubular Carcinoma

5.              Invasive Mammary Carcinoma, NOS (ductal, lobular, mixed)

 

 

 

S100

CK

EMA

ER

Myoepithelial

Markers

Basement Membrane

MGA&AMGA

++

-/+

-

-

-

+

Adenoid Cystic CA

+

+

+

-

+

+

Tubular CA

-

++

++

++

-

-

Mammary NOS

-/+

++

++

+/-

-

-

 

Final Diagnosis:

Invasive Mammary carcinoma arising in microglandular adenosis

 

References:

1.              James BA, Cranor ML, Rosen PP. Carcinoma of the breast arising in Microglandular Adenosis.  American Journal of Clinical Pathology 1993:100-507-13.

2.              Rosen PP. Microglandular adenosis: A benign lesion simulating invasive mammary carcinoma.  American Journal of Surgical Pathology 1983:7:137-44.

3.              Acs G, Simpson J, Bleiweiss I, Hugh J, Reynolds C, Olson S, Page D.  Microglandular Adenosis with transition into adenoid cystic carcinoma of the breast. The American Journal of Surgical Pathology. 2003:8:1052-60.

4.              Koenig C, Dadmanesh F, Bratthauer G, Tavassoli F. Carcinoma arising in             Microglandular adenosis: An immunohistochemical analysis of 20 intraepithelial             and invasive neoplasms.  International Journal of Surgical pathology 8(4):303-            315,2000.

 

Case# 6:  Srinidhi Musunuri, M.D

 

History: 

¤                48 y.o. male presented to an outside hospital with acute flank pain.

¤                CT scan showed a large left perirenal hematoma and a possible renal mass. Exploratory laparotomy was performed and a friable hemorrhagic mass in the upper pole of the left kidney was removed in fragments followed by nephrectomy and a diagnosis of granular variant of RCC was rendered. The patient came to Northwestern for further treatment

 

Histology:

¤                Polygonal cells with clear to eosinophilic cytoplasm arranged in sheets and nests separated by thin fibrovascular septae. Some of the cells have abundant eosinophilic cytoplasm with eccentric nuclei giving a strap like appearance to the cells. Focally the cells are spindled. Some entrapped tubules and rare thick walled blood vessels seen

 

Differential diagnosis

¤                Epithelioid angiomyolipoma

¤                Renal cell carcionoma

 

   Immunohistochemistry

¤                Tumor cells are focally positive for HMB 45 and negative for AE1/ AE3 and EMA

 

      Diagnosis

¤                Epithelioid Angiomyolipoma

 

      Reference

.

¤                Desai S. Hejmadi R. Krishnamurthy S. Chinoy RF. Renal angiomyolipoma. A clinicopathologic, immunohistochemical, and follow-up study of 46 cases. AJSP. 25(7):972-3, 2001 Jul.

¤                Eble JN. Amin MB. Young RH. Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component. AJSP 21(10):1123-30, 1997 Oct.

¤                Sean K. Lau, Alberto M. Marchevsky, Robert J. McKenna Jr and Daniel J. Luthringer,  Malignant Monotypic Epithelioid Angiomyolipoma of the retroperitoneumÓ, International Journal of Surgical Pathology

¤                Cibas ES. Goss GA. Kulke MH. Demetri GD. Fletcher CD. Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney. AJSP. 25(1):121-6, 2001 Jan.

¤                Pea M. Martignoni G. Zamboni G. Bonetti F. Perivascular epithelioid cell.[comment]. AJSP. 20(9):1149-53, 1996 Sep.

 

 

 

 

Case #7 - Kurt T. Patton, MD

 

History: The patient is an otherwise healthy 51 year-old male who presented with small bowel obstruction who underwent subsequent resection.

 

Gross

á               Small bowel with a 3 X 3 X 3 cm firm, smooth, pedunculated mass showing focal mucosal ulceration.

á               Cut surface is homogenous, tan-white, with stranding.

á               Well-circumscribed, expanding the lamina propria.

á               Hemorrhage and necrosis is absent.

 

Histology

á               Low magnification, lesion arises from the muscularis propria, well circumscribed.

á               At higher magnification, moderately cellular proliferation of spindle and stellate cells lying in a wavy, wire-like collagenous matirx.

á               Poorly formed fascicles focally.

á               Keloid-type collagen is present in some areas.

á               In other areas the lesional cells lie within a myxoid matrix.

 

Differential Diagnosis

á               Inflammatory fibroid polyp

á               Gastrointestinal stromal tumor (GIST)

á               Intra-abdominal (desmoid) fibromatosis

á               Inflammatory myofibroblastic tumor

á               Reactive nodular fibrous pseudotumor

 

Immunohistochemical profile

á               Stains for CD117 (c-kit) and CD34 are negative

 

Diagnosis: Reactive nodular fibrous pseudotumor

 

References

 

1.              Yantiss RK et al. AJSP 27(4): 532-540, 2003.

2.              Riddell RH et al. Tumors of the intestines, AFIP Atlas of tumor pathology, third series.

3.              Kempson RL et al. Tumors of the soft tissues, AFIP Atlas of tumor pathology, third series.

4.              Yantiss RK et al. AJSP 24(7): 947-957, 2000.