Large epithelioid tumours

From Libre Pathology
Jump to: navigation, search
A large epithelioid tumour (epithelioid angiosarcoma). H&E stain.

Large epithelioid tumours are common and represent a large differential diagnosis when viewed generally.

Malignant epithelioid lesions are often carcinomas... but may be a large number different tumours.

A more general discussion of epithelioid lesions is in the epithelioid cell lesions article. Small epithelioid lesions are dealt with in the small round cell tumours article.

General differential diagnosis

Adapted from Miller with modifications:[1]

Tumour Group Notes/comments
Melanoma Miscellaneous
PEComa Miscellaneous esp. epithelioid angiomyolipoma
Perivascular epithelioid cell sarcoma Miscellaneous type of PEComa, looks like clear cell carcinoma[2]
Paraganglioma-like dermal melanocytic tumour Miscellaneous [3]
Carcinoma Epithelial tumours HCC and adrenal cortical carcinoma, often AE1/AE3 -ve
Hepatoid Adenocarcinoma Epithelial tumours
Malignant mixed Muellerian tumour Epithelial tumours
Myoepithelial carcinoma Epithelial tumours
Myoepithelioma of soft tissue Epithelial tumours may mimic extraskeletal myxoid chondrosarcoma or proximal variant of epithelioid sarcoma
Acinar cell carcinoma Epithelial tumours
Neuroendocrine carcinoma Epithelial tumours may have a rhabdoid morphology[4]
Medullary thyroid carcinoma Epithelial tumours
Thymoma Epithelial tumours
Cutaneous myoepithelioma Epithelial tumours [5]
Cutaneous adnexal tumours (spiradenoma, acrospiroma) Epithelial tumours
Hyaline-rich chondroid syringoma Epithelial tumours
Hurthle cell tumour Epithelial tumours
Mesothelioma Epithelial tumours "lymphohistiocytoid" variant
Seminoma Germ- cell / Sex-Cord Stromal
Embryonal carcinoma Germ- cell / Sex-Cord Stromal
Yolk sac tumour Germ- cell / Sex-Cord Stromal consider hepatoid variant
Choriocarcinoma Germ- cell / Sex-Cord Stromal
Epithelioid trophoblastic tumour Germ- cell / Sex-Cord Stromal
Granulosa cell tumour Germ- cell / Sex-Cord Stromal
Endometrial stromal sarcoma Germ- cell / Sex-Cord Stromal high grade
Lipid cell tumour of ovary Germ- cell / Sex-Cord Stromal
Sertoli cell tumour Germ- cell / Sex-Cord Stromal also Sertoli-Leydig cell tumour
Leydig cell tumour Germ- cell / Sex-Cord Stromal also Sertoli-Leydig cell tumour
Epithelioid trophoblastic tumour Germ- cell / Sex-Cord Stromal [6][7]
Placental site trophoblastic tumour Germ- cell / Sex-Cord Stromal (USCAP04, Gyn spec conf, case 3, vaginal
Testicular tumour of the adrenogenital syndrome Germ- cell / Sex-Cord Stromal Inhibin -ve, dark brown-green-black, similar to Leydig tumour
Extrauterine lesion of intermediate trophoblast Germ- cell / Sex-Cord Stromal very rare
Lymphoma Hematolymphoid / Histiocytic tumours
Hodgkin's lymphoma Hematolymphoid / Histiocytic tumours syncytial or lymphocyte-depleted
Extramedullary monoblastic leukemia Hematolymphoid / Histiocytic tumours
Histiocytic sarcoma Hematolymphoid / Histiocytic tumours "True" histiocytic lymphoma
Langerhans cell histiocytosis Hematolymphoid / Histiocytic tumours
Rosai-Dorfman disease Hematolymphoid / Histiocytic tumours
Crystal-storing histiocytosis Hematolymphoid / Histiocytic tumours
Erdheim-Chester disease Hematolymphoid / Histiocytic tumours foamy histiocytes
Rhinoscleroma Hematolymphoid / Histiocytic tumours foamy histiocytes and chronic inflammation
Granuloma annulare Hematolymphoid / Histiocytic tumours
Anaplastic myeloma Hematolymphoid / Histiocytic tumours
Extramedullary myeloid leukemia Hematolymphoid / Histiocytic tumours
Extramedullary lymphoid leukemia Hematolymphoid / Histiocytic tumours
Extramedullary megakaryoblastic leukemia Hematolymphoid / Histiocytic tumours
Erythroleukemia Hematolymphoid / Histiocytic tumours may be EMA +ve
Juvenile xanthogranuloma Hematolymphoid / Histiocytic tumours
Paraganglioma Neural - Neuroendocrine - Neuraxis Tumours also pheochromocytoma
High grade olfactory neuroblastoma Neural - Neuroendocrine - Neuraxis Tumours
Epithelioid MPNST Neural - Neuroendocrine - Neuraxis Tumours reported to have focal melanocytic differentiation in some cases[citation needed]
Glioblastoma Neural - Neuroendocrine - Neuraxis Tumours
Epithelioid schwannoma / neurofibroma Neural - Neuroendocrine - Neuraxis Tumours
Benign epithelioid nerve sheath tumour Neural - Neuroendocrine - Neuraxis Tumours
Chordoma Neural - Neuroendocrine - Neuraxis Tumours
Meningioma Neural - Neuroendocrine - Neuraxis Tumours chordoid meningioma
Parachordoma Neural - Neuroendocrine - Neuraxis Tumours [8]
Ependymoma Neural - Neuroendocrine - Neuraxis Tumours
Epithelioid perineurioma Neural - Neuroendocrine - Neuraxis Tumours
Hemangioblastoma Neural - Neuroendocrine - Neuraxis Tumours may be extraneural
Rhabdoid tumour Neural - Neuroendocrine - Neuraxis Tumours INI1 -ve
Mucosal epithelioid nerve sheath tumour Neural - Neuroendocrine - Neuraxis Tumours
Neurothekeoma Neural - Neuroendocrine - Neuraxis Tumours
Rhabdomyosarcoma, not otherwise specified (pleomorphic rhabdomyosarcoma) Muscle-related tumours
Epithelioid leiomyosarcoma Muscle-related tumours
Epithelioid glomus tumour Muscle-related tumours
Granular cell tumour Muscle-related tumours neural, non-neural types
Rhabdomyoma Muscle-related tumours
Myopericytoma Muscle-related tumours [9]
Epithelioid angiosarcoma Vascular tumours
Epithelioid hemangioendothelioma Vascular tumours
Epithelioid sarcoma-like hemangioendothelioma Vascular tumours [10]
Composite hemangioendothelioma Vascular tumours
Hemangiopericytoma Vascular tumours
Cutaneous epithelioid angiomatous nodule Vascular tumours
Bacillary angiomatosis Vascular tumours
Malignant pigmented villonodular synovitis Hematolymphoid / Histiocytic tumours
Multicentric Reticulohistiocytosis Hematolymphoid / Histiocytic tumours
Follicular dendritic cell sarcoma Hematolymphoid / Histiocytic tumours
Interdigitating reticulum cell sarcoma Hematolymphoid / Histiocytic tumours S-100 +ve
Diffuse type tenosynovial giant cell tumour Hematolymphoid / Histiocytic tumours
Malakoplakia Hematolymphoid / Histiocytic tumours
Kikuchi's disease Hematolymphoid / Histiocytic tumours
Xanthogranuloma Hematolymphoid / Histiocytic tumours
Epithelioid sarcoma Miscellaneous mesencchymal tumours especially proximal variant
Synovial sarcoma Miscellaneous mesencchymal tumours poorly differentiated
Alveolar soft part sarcoma Miscellaneous mesencchymal tumours TFE3 +ve
Gastrointestinal stromal tumour Miscellaneous mesencchymal tumours
Round cell liposarcoma Miscellaneous mesencchymal tumours
Dedifferentiated liposarcoma Miscellaneous mesencchymal tumours
Epithelioid variant of pleomorphic liposarcoma Miscellaneous mesencchymal tumours
Polyphenotypic small cell tumour Miscellaneous mesencchymal tumours
Ewing Sarcoma / PNET, large cell type Miscellaneous mesencchymal tumours
Epithelioid fibrous histiocytoma Myofibroblastic - fibroblastic tumours
Inflammatory malignant fibrous histiocytoma Myofibroblastic - fibroblastic tumours
Sclerosing epithelioid fibrosarcoma Myofibroblastic - fibroblastic tumours
Epithlelioid myofibroblastoma Myofibroblastic - fibroblastic tumours
Angiomatoid malignant fibrous histiocytoma Myofibroblastic - fibroblastic tumours
Inflammatory myxohyaline tumour of distal extremities with virocyte or Reed-Sternberg like cells Myofibroblastic - fibroblastic tumours
Acral myxoinflammatory fibroblastic sarcoma Myofibroblastic - fibroblastic tumours
Angiomyofibroblastoma Myofibroblastic - fibroblastic tumours
Epithelioid osteosarcoma Chondro-osseous tumours [11]
Extraskeletal myxoid chondrosarcoma, high grade Chondro-osseous tumours
Epithelioid multinodular osteoblastoma Chondro-osseous tumours may mimic osteosarcoma[12]
Radiation effect Miscellaneous

See also


  1. Miller RT. (Sep 2013). Atlantic Diagnostic Immunohistochemistry Symposium. St. John's, NL, Canada.
  2. Bonetti, F.; Martignoni, G.; Colato, C.; Manfrin, E.; Gambacorta, M.; Faleri, M.; Bacchi, C.; Sin, VC. et al. (Jun 2001). "Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.". Mod Pathol 14 (6): 563-8. doi:10.1038/modpathol.3880351. PMID 11406657.
  3. Deyrup, AT.; Althof, P.; Zhou, M.; Morgan, M.; Solomon, AR.; Bridge, JA.; Weiss, SW. (Dec 2004). "Paraganglioma-like dermal melanocytic tumor: a unique entity distinct from cellular blue nevus, clear cell sarcoma, and cutaneous melanoma.". Am J Surg Pathol 28 (12): 1579-86. PMID 15577676.
  4. Perez-Montiel, MD.; Frankel, WL.; Suster, S. (May 2003). "Neuroendocrine carcinomas of the pancreas with 'Rhabdoid' features.". Am J Surg Pathol 27 (5): 642-9. PMID 12717248.
  5. Kutzner, H.; Mentzel, T.; Kaddu, S.; Soares, LM.; Sangueza, OP.; Requena, L. (Mar 2001). "Cutaneous myoepithelioma: an under-recognized cutaneous neoplasm composed of myoepithelial cells.". Am J Surg Pathol 25 (3): 348-55. PMID 11224605.
  6. Hamazaki, S.; Nakamoto, S.; Okino, T.; Tsukayama, C.; Mori, M.; Taguchi, K.; Okada, S. (Nov 1999). "Epithelioid trophoblastic tumor: morphological and immunohistochemical study of three lung lesions.". Hum Pathol 30 (11): 1321-7. PMID 10571512.
  7. Shih, IM.; Kurman, RJ. (Nov 1998). "Epithelioid trophoblastic tumor: a neoplasm distinct from choriocarcinoma and placental site trophoblastic tumor simulating carcinoma.". Am J Surg Pathol 22 (11): 1393-403. PMID 9808132.
  8. Folpe, AL.; Agoff, SN.; Willis, J.; Weiss, SW. (Sep 1999). "Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma.". Am J Surg Pathol 23 (9): 1059-67. PMID 10478665.
  9. Mentzel, T.; Dei Tos, AP.; Sapi, Z.; Kutzner, H. (Jan 2006). "Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases.". Am J Surg Pathol 30 (1): 104-13. PMID 16330949.
  10. Billings, SD.; Folpe, AL.; Weiss, SW. (Jan 2003). "Epithelioid sarcoma-like hemangioendothelioma.". Am J Surg Pathol 27 (1): 48-57. PMID 12502927.
  11. Amanatullah, DF.; Ngann, KK.; Borys, D.; Tamurian, RM. (Jun 2010). "Progression of aggressive metastatic carcinosarcoma after treatment of epithelioid osteosarcoma.". Orthopedics 33 (6): 445. doi:10.3928/01477447-20100429-26. PMID 20806764.
  12. Zon Filippi, R.; Swee, RG.; Krishnan Unni, K. (Aug 2007). "Epithelioid multinodular osteoblastoma: a clinicopathologic analysis of 26 cases.". Am J Surg Pathol 31 (8): 1265-8. doi:10.1097/PAS.0b013e31803402e7. PMID 17667552.