John H. Stroger, Jr. Hospital of Cook County

Illinois Registry of Anatomic Pathology

April 26, 2004

 

 

 

CASE 1

 

Resident:        Jyothi Patil, MD

Attending:        Paula Kovarik, MD

 

Diagnosis:       Angioimmunoblastic T-Cell Lymphoma With Large B Cell                                                          Expansion, EBV-Related

 

Clinical history: 54 years old male with fairly rapid onset of cervical followed by generalized lymphadenopathy

 

Gross Examination: Lymph node measuring 2.0x1.6x1.6 cm, with homogenous pink-tan cut surface

 

Microscopic Examination: The architecture of the lymph node is effaced. The node is replaced by a diffuse lymphoid infiltrate consisting of immunoblasts, plasma cells, and small lymphocytes. There are prominent arborizing venules present. Many areas show cluster of cells with clear cytoplasm.

 

Immunohistochemistry: Majority of the cells are CD 3 positive T-cells, CD 20 stains numerous B cells and also large cells with open chromatin. C10 negative. Insitu hybridization showed scattered positive cells.

 

Flow cytometry: Reduced number of CD7 cells.

 

Summary: Angioimmunoblastic T-cell lymphoma is a relatively rare in the western countries, accounting for 1-2% of non-HodgkinÕs lymphoma. The clinical course is aggressive with median survival of three years.

 

References:

  1. Elaine S. Jaffe et al. World Health Organization Classification of Tumors
  2. Attygalle A, al-Jehani R et al. Neoplastic T-cells in Angioimmunoblastic T-Cell Lymphoma Express CD10. Blood Jan 15;99(2):627-33. 2000.
  3. Lome-Maldonado, et al. ÒAngioimmunoblastic T-cell lymphoma (AILD-TL) rich in large B-cells and associated with Epstein-Barr virus infection. A different subtype of AILD-TLÓ. Leukemia 16(10) 2134-2141. 2002.
  4. Attygalle A. et al. C10 Expression in Extranodal Dissemination of Angioiimmunoblastic T-cell Lymphoma. Am J Surg Pathol 28:54-61. 2004
  5. Andres Zettl et al. Epstein-Barr Virus-Associated B-cell Lymphoproliferative Disorders in Angioimmunoblastic T-cell Lymphoma and Peripheral T-cell Lymphoma, Unspecified. Am J Clin Pathol 117:368-379. 2002.
  6. Dogan et al. Angioimmunoblastic T-Cell Lymphoma, Review. Br J Haem 121, 681-691. 2003
  7. Yin Xu et l. Composite Angioimmunoblastic T-cell Lymphoma and Diffuse Large B-cell Lymphoma. Am J Clin Pathol 118:848-854. 2002.

 

CASE 2

Resident: Cho Lwin, MD

Attending: Gabor Tarjan, MD

 

Diagnosis: Omental Epithelioid Extragastrointestinal Stromal Tumor

Differential Diagnosis:

1)     Metastasis from colonic primary

2)     Paraganglioma

3)     Ectopic adrenocortical tissue

4)     Pseudotumoral deciduosis

5)     Deciduoid mesothelioma

6)     Plasmacytoma

7)     Neoplasms with intranuclear pseudoinclusions

Hepatocellular carcinoma

Lung (bronchioloalveolar) carcinoma

Papillary thyroid carcinoma

Melanoma

Gross Examination: Nodular or multinodular mass, usually with solid tan cut surface.

Microscopic Examination: Sheets of relatively uniform, mostly round cells with eosinophilic cytoplasm, well-defined cell borders, and central or eccentric nuclei.. Similar but elongated spindle cells may be seen focally. In our case, some cells demonstrated prominent intranuclear pseudoinclusions. These have been mentioned in a cytology report but have not been emphasized in the surgical pathology literature.

Immunohistochemistry: The tumor cells are positive for CD 117 (c-kit), CD 34, and may show focal SMA positivity.

 

Clinical features:

Omental extragastrointestinal stromal tumors are rare and the number of reported cases are limited. They are usually found incidentally during surgery or other workup.

Most omental EGISTs are large (>10 cm). In these cases, the prognosis correlates with cellularity, mitotic activity, and necrosis. Rare small omental EGISTs have excellent prognosis.

 

References:

1. Miettinen et al. Gastrointestinal Stromal Tumors/Smooth Muscle Tumors (GISTs) Primary in the Omentum and Mesentery: Clinicopathologic and Immunohistochemical Study of 26 Cases. Am J Surg Path 23:1109, 1999.

2.     John D. Reith, Sharon W. Weiss et al. Extragastrointestinal (Soft Tissue) Stromal Tumors: An Analysis of 48 Cases with Emphasis on Histologic Predictors of Outcome. Mod Path 13:577, 2000

3.     Miettinen et al. Gastrointestinal Stromal Tumors-Definition, Clinical, Histological, Immunohistochemical, and Molecular Genetic Features and

Differential Diagnosis: Virch Arch 438:1, 2001.

4.     Qun Dong et al. Epithelioid Variant of Gastrointestinal Stromal Tumor: Diagnosis by Fine-Needle Aspiration. Diag Cytopath, 29:55, 2003.

5.     Nakagawa et al. Extragastrointestinal Stromal Tumor of the Greater Omentum: Hepato-Gastroenterology 50:691, 2003.

6.     Greenson et al. Gastrointestinal Stromal Tumors and Other Mesenchymal Lesions of the Gut: Mod Path 16:366, 2003.

 

 

 

CASE 3

 

 

Resident: Feinan Shi, MD

Attending: Amila Orucevic, MD, PhD

 

 

Diagnosis: Mucinous cystic tumor of borderline malignant potential with pseudoneoplastic                  proliferation of endocrine cells

 

 

Differential diagnosis for the cystic lesion:

1. Intraductal papillary mucinous tumor (with cystic dilatation)

2      Mucinous cystic tumor

-        Adenoma

-        Borderline malignant potential

-        Cystadenocarcinoma

 

 

Differential diagnosis for the exuberant endocrine proliferation:

1.     Endocrine neoplasia

2.     Pseudoneoplastic proliferation of endocrine cells

 

 

Key Pathologic Features

¤       A multiloculated, cystic lesion (4.5 x 4.5 x 2.5 cm) containing hemorrhagic mucinous fluid is seen in the pancreatic body / tail region on gross examination.

 

¤       Microscopic examination of the entire lesion shows cysts lined by a single layer of tall columnar mucinous cells with basally oriented nuclei. There is only a focal epithelial pseudo-stratification with moderate nuclear atypia, compatible with borderline malignant potential of this mucinous cystic tumor.

 

¤       Exuberant endocrine proliferation is seen only in the tissue surrounding the cystic lesion as an incidental finding on microscopic examination. The proliferating individual islets are normal in size and shape.

 

¤       Immunocytochemical studies show endocrine cells in the proliferating islets to be positive for insulin, glucagon and somatostatin. These cells are normal in distribution and proportion, which is compatible with the pseudoneoplastic nature of the process.

 

 

References:

1.     Bartow SA, Mukai K, Rosai J. Pseudoneoplastic Proliferation of Endocrine Cells in Pancreatic Fibrosis. 1981; Cancer 47: 2627-2633

2.     Wilentz RE, Albores-Saavedra J, Hruban RH. Mucinous Cystic Neoplasms of the Pancreas. 2000; Semin Diagn Pathol 17(1): 31-42

3.     Oertel E. The Pancreas: Nonneoplastic Alterations. 1989; Am J

Surg Pathol 13 (Suppl. 1): 50-65.

 

 

 

 

CASE 4

 

 

Resident: Shekar Puttaswamy, M.D.

Attending: Rajyasree Emmadi, M.D.

 

 

Diagnosis: Anaplastic Rhabdomyosarcoma

 

 

Differential diagnosis:

1. MPNST

2. Epithelioid Sarcoma

3. Undifferentiated Sarcoma

4. Rhabdomyosarcoma

 

 

Clinical history: A 3-year-old child was brought to E.R for an enlarging left shoulder mass. Imaging studies revealed a solid heterogenous soft tissue mass with possible areas of necrosis.

 

Key Pathologic Features:

Morphology: Sheets of spindle cells with a hyalinized stroma. There were numerous mitotic figures and necrosis.

Immunohistochemistry: The malignant cells were positive for desmin, myogenin and muscle specific actin.

 

Summary: A diagnosis of anaplastic rhabdomyosarcoma was made based on morphology and immunohistochemistry. The lesion showed features of anaplasia as defined by the WilmÕs tumor classification i.e., 3-fold increase in nuclear size compared to the adjacent malignant cell nuclei and if anaplasia is diffuse, it portends a poor prognosis.

 

References:

1.     Coffin CM. The New International Rhabdomyosarcoma Classification, Its Progenitors, and Considerations Beyond Morphology. Advances in Anatomic Pathology 1997; 4:1-16.

2.     Kodet R, Newton WA, Hamoudi AB et al. Childhood Rhabdomyosarcoma With Anaplastic (Pleomorphic) Features: A Report of the Intergroup Rhabdomyosarcoma Study. American Journal of Surgical Pathology 1993; 17:443-453.

3.     Qualman SJ, Coffin CM, Newton WA, et al. Intergroup Rhabdomyosarcoma Study: Update for Pathologists. Pediatric and Developmental Pathology 1998; 1:550-561.

 

 

 

 

 

 

 

CASE 5

 

Resident:         Songlin Zhang, MD, PhD

Attending:        Bourke Firfer, MD, MPH

 

Diagnosis:       Renal Medullary Carcinoma

 

Differential Diagnosis:

o      Metastatic adenocarcinoma.

o      Adenocarcinoma of renal pelvic mucosa.

o      Renal papillary carcinoma.

o      High grade transitional cell carcinoma.

o      Juxtaglomerular cell tumor

o      Collecting ductal carcinoma.

o      Renal medullary carcinoma

 

Clinical History : The patient was a 31 year-old African-American male with two and half month-history of low back pain. CT and MRI showed an L3 vertebral lytic lesion, a liver lesion, and a renal mass. The rest of the medical history is unremarkable.

 

 

Key Pathologic Features:

 

Gross: One demarcated solid mass was located in the medulla and cortex of the right kidney measuring 3.0 cm in the greatest dimension with marked central necrosis and satellite lesions. The renal pelvis was unremarkable.

 

Microscopic: The renal lesion shows a solid, reticular pattern with marked inflammatory reaction, extensive necrosis, and desmoplastic stroma. The tumor cells have high grade nuclei and prominent nucleoli. Signet ring cells are present. The metastatic lesions in the bone and lungs have a similar morphology. The hepatic lesion is a hemangioma.

 

 

Immunohistochemistry: The tumor cells are reactive with antibodies to pancytokeratin, vimentin, EMA, polyclonal CEA. They are nonreactive with CK7, CK20, high molecular weight cytokeratin, and neuroendocrine markers.

 

References:

1. Davis CJJ et al: Renal Medullary Carcinoma: The Seventh Sickle Cell Nephropathy.

Am J Surg Pathol 19:1-11, 1995.

2. Norman R. et al: Histopathology and Molecular Genetics of Renal Tumors: Toward

Unification of A Classification System. J Urol 162:1246-1258, 1999.

3. Srigley JR and JN Eble: Collecting Duct Carcinoma of Kidney. Sem Diagn Path 15:54-67,

1998.

4. Swartz MA et al: Renal Medullary Carcinoma: Clinical, Pathologic, Immunohistochemical,

and Genetic Analysis with Pathogenetic Implications. Urology 60:1083-1089, 2002.

5. Thomas J. et al: Molecular Genetics and Histopathological Features of Adult Distal

Nephron Tumors. Urology 60:941-946, 2002.

6. Wesche WA et al: Renal Medullary Carcinoma: A Potential Sickle Cell Nephropathy of

Children and Adolescents. Ped Path Lab Med 18:97-113, 1998.

7. Yang X et al: Gene Expression Profiling of Renal Medullary Carcinoma. Cancer 100:976-

985, 2004.

 

CASE 6

 

Resident:         Xi Wang, MD PhD

Attending:        Stephanie Young, MD

 

Diagnosis:       Clear Cell Sarcoma

 

Differential Diagnoses:

  1. Metastatic Melanoma
  2. Epithelioid Sarcoma
  3. Synovial Sarcoma
  4. Alveolar Rhabdomyosarcoma

 

Introduction: This is a rare soft tissue sarcoma that predominantly occurs in extremities, though, as in our case, it can occur in the trunk. Treatment is usually surgical removal. Metastasis may occur after a long tumor-free period, with lung as the predominant metastatic location. Sentinel lymph node examination has been recommended.

 

Gross Examination: The tumor is poorly circumscribed. The cut surface of tumor is fleshy and variegated with focal hemorrhage.

Microscopic Examination: In most areas, the neoplastic cells are uniformly epithelioid and divided by fibrous septa. In focal areas, cells are spindle-shaped, whereas in other areas, cells form an organoid pattern and some exhibit brown pigment.

 

Immunohistochemistry: The neoplastic cells are positive for HMB45, S-100 and vimentin, but negative for pancytokeratin, EMA, actin, and desmin.

 

Cytogenetics: Our tumor has a characteristic chromosomal translocation with t(12;22)(q13;q12).

 

References:

    1. Kempson, RL, et al: Atlas of Tumor Pathology Tumors of Soft Tissue. AFIP 2001 463-467.
    2. Weiss and Goldblum: Enzinger and WeissÕs Soft Tissue Tumors. Fourth Ed. 2001 1241-1250.Okamura, N., et al: Case report: Case of Clear Cell Sarcoma Surviving With the Primary Lesion for 20 years After Resection of a Metastatic Lymph Node. Anti-cancer Research 2003 23(5b): 4197-4203.
    3. Parwani, AV, et al: Pathologic Quiz Case. Arch Pathol Lab Med 2004 128: e56-e57.
    4. Teixeira, MR, et al: Karyotypic Divergence and Convergence in Two Synchronous Lung Metastases of a Clear Cell Sarcoma of Tendons and Aponeuroses With t(12;22)(q13;q12) and Type 1 EWS/ATF1. Cancer Genet Cytogenet. 2003 145(2): 121-125.
    5. Antonescu, CR, et al Molecular Diagnosis of Clear Cell Sarcoma. J. Mol. Diag. 4: 44-52..

 

CASE 7

 

Fellow:            Reuben Cuison, M.D.

Attendings:      Ephraim Axelrod, M.D. and Marin Sekosan, M.D.

 

Diagnosis:       Inflammatory myofibroblastic tumor (a.k.a. Inflammatory pseudotumor, Plasma                                     cell granuloma) in the left pulmonary artery

 

Differential Diagnosis:

            1. Extramedullary plasmacytoma

            2. Pulmonary artery sarcoma

            3. Inflammatory fibrosarcoma

 

Key Features: Inflammatory myofibroblastic tumors (IMTs) are uncommon lesions that occur most often in the lung; also reported to occur in virtually all major areas of the body, especially in the peritoneum and retroperitoneum. Primary or secondary occurrence in the pulmonary artery has never been reported.

            It has traditionally been thought of as arising in an inflammatory setting although a neoplastic origin for some cases has been been established. This is thus still a fairly heterogeneous group of lesions, having the myofibroblast as the principal cell type, perhaps best considered as representing a spectrum of fibroinflammatory lesions, some neoplastic, arising in various anatomic sites with varying etiologies and biologic behavior.

            Conservative surgery is mainstay of therapy with excellent prognosis for completely resected lesions. There are occasional reports of pulmonary IMTs with infiltration of adjacent structures and rare reports of recurrence, metastasis and malignant transformation.

            Best to designate this as having an uncertain malignant potential.

 

Gross Examination: Usually 1 to 6 cm well-circumscribed intrapulmonary firm tan-white nodule. Occasionally calcified but hemorrhage and necrosis unusual. Our case was entirely within the left pulmonary artery with marked thickening of arterial wall.

 

Microscopic Examination: Mostly bland spindle cells (fibroblasts and myofibroblasts) in various patterns with variable but usually significant lymphoplasmacytic infiltrate, occasional xanthomatous foci and sclerotic areas. Mitotic activity is low.

 

Immunohistochemistry: Spindle cells are diffusely positive for vimentin, focally positive for actin (smooth muscle actin or muscle specific actin), desmin and cytokeratin, and typically negative for S-100. Plasma cells are polyclonal.

 

Cytogenetics: Various clonal chromosomal aberrations have been reported in some cases of IMTs, the most frequent being translocations involving chromosome 2p23 and ALK1 gene.

 

References:

  1. Burke AP, Virmani R. Sarcomas of the pulmonary artery. In: Atlas of Tumor Pathology-Tumors of the Heart and Great Vessels. Washington, DC: AFIP, 1996:217-221.
  2. Cessna MH, Zhou H, Sanger WG, Perkins SL, Tripp S, Pickering D, Daines C, Coffin CM. Expression of ALK1 and p80 in Inflammatory Myofibroblastic Tumor and its Mesenchymal Mimics: A Study of 135 cases. Mod Pathol 2002;15:931-938.
  3. Coffin CM, Dehner LP, Meis-Kindblom JM. Inflammatory Myofibroblastic Tumor, Inflammatory Fibrosarcoma, and Related Lesions: A Historical Review with Differential Diagnostic Considerations. Semin Diagn Pathol 1998;15:102-110.
  4. Coffin CM, Patel A, Perkins S, Elenitoba-Johnson KSJ, Perlman E, Griffin CA. ALK1 and p80 Expression and Chromosomal Rearrangements Involving 2p23 in Inflammatory Myofibroblastic Tumor. Mod Pathol 2001;14:569-576.
  5. Travis WD, Colby TV, Koss MN, Rosado-de-Christenson ML, Muller NL, King TE. Non-neoplastic Disorders of the Lower Respiratory Tract. First Series/Fascicle 2 of Atlas of Nontumor Pathology. Washington, DC: The American Registry of Pathology;2002:857-866.
  6. Meis JM, Enzinger FM. Inflammatory Fibrosarcoma of the Mesentery and Retroperitoneum. A Tumor Closely Simulating Inflammatory Pseudotumor. Am J Surg Pathol 1991;15:1146-1156.
  7. Pettinato G, Manivel JC, De Rosa N, Dehner LP. Inflammatory Myofibroblastic Tumor (Plasma Cell Granuloma). Clinicopathologic Study of 20 cases With Immunohistochemical and Ultrastructural Observations. Am J Clin Pathol 1990;94:538-546.