epithelioid sarcoma ppt

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rare cases from journals... sarcomas of orbit

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JOURNAL CLUB 1. Epithelioid Sarcoma of the Orbit: A Case report 2. Fibrous Histiocytoma of Orbit : A Case Report PRESENTER -- Dr. Ravneet Kaur MODERATOR – Dr. Manjit Singh Bal

Epithelioid Sarcoma of the Orbit: A Case report Hind M.Alkatan, Imtiaz Chaudhry, Abdullah Al-Qahtani Department of Pathology and Laboratory Medicine, Oculoplastic and Orbital Division, King Khaled Eye Specialist Hospital, Saudi Arabia Ann Saudi Med 2011:31(2):187-189 :

Epithelioid Sarcoma of the Orbit: A Case report Hind M.Alkatan , Imtiaz Chaudhry , Abdullah Al- Qahtani Department of Pathology and Laboratory Medicine, Oculoplastic and Orbital D ivision, King Khaled Eye Specialist Hospital, Saudi Arabia Ann Saudi Med 2011:31(2):187-189 CASE - 1

Clinical presentation:

Clinical presentation 5 month, male H/O proptosis R eye x 1 month On Exam : severe proptosis , conjuctival chemosis due to a supra-orbital mass Preoperative Investigations : MRI revealed 3.4 x 2.6 cm well defined mass with cystic changes Right orbital biopsy was performed Initial diagnosis : Lymphangioma

Histopathology:

Histopathology Multinodular pattern of growth Large pleomorphic epithelioid cells, abundant cytoplasm, vesicular nuclei and prominent nucleoli Necrosis Frequent Mitotic Figures

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Moderate positivity with cytokeratin Negative to desmin and myogenin CD34 VIMENTIN

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Diagnosis : Epithelioid sarcoma “ proximal type” Mx of patient: Debulking of tumor + 3 # of CT No response exenteration at age of 7 months Outcome : Child died at 9 months due to intracranial extension of recurrence.

Discussion :

Discussion Rare malignant tumor Enzinger in 1970 reported a large series of 241 cases Two types : “Distal” type “Proximal” type Distal : young males, distal upper extremity Proximal : older adults, pelvis, perineum, genital tract

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Histogenesis : - uncertain , Head and neck rarely involved Primary orbital epithelioid sarcoma : 2 cases reported in 1993 - pseudogranulomatous appearance due to central necrosis - polygonal to spindle cells with vesicular nuclei and abundant eosinophilic cytoplasm - strong vimentin staining, moderate for keratin and negative for desmin , S100 and myoglobin

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Prognosis : Not favourable Local recurrence in 77% and 45% to lymph nodes and lungs Recurrence depends on adequacy of initial excision

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Adverse prognostic indicators - Male - Older age - Large tumor size - Deep location Vascular invasion Nerve invasion Orbital disease has relentless progression.

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Early detection, proper histopathological diagnosis and adequate surgical excision are of immense importance

Epithelioid Sarcoma:

Epithelioid Sarcoma Extremely rare, Aggressive malignancy Slow growing tumor with a high rate of recurrence and metastasis Etiology of ES is obscure Inactivation of the SMARCB1/INI1 tumor suppressor gene has been identified in roughly 85% of tumors Located on long arm of chromosome 22 Tumor of uncertain histogenesis

Variants:

Usually - adolescents & young adults Male : Female (1.8:1) Most common primary site for epithelioid sarcoma is distal upper extremities like hands and forearms However, ES have been reported in vulva, penis and spine Distal – classic/ conventional Proximal Variants

Presentation :

Presentation Most tumors present as firm-to-hard palpable masses, either in the deep soft tissue or in the dermis Superficial lesions will ulcerate Deep tumors can be located in fascial planes, aponeurosis and tendon sheaths Usually painless and slow growing

Diagnosis :

Diagnosis MRI - diagnostic modality of choice for imaging prior to biopsy and pathologic diagnosis Tissue biopsy- confirmatory

Histopathology:

Histopathology Gross – one or more white nodules with infiltrating margins Nodular nature is characteristic of ES

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Tumour consists – epithelial appearing (ovoid or polygonal) cells well blended with fusiform cells which are strongly eosinophilic Necrosis often appears in the center of nodules Extensive desmoplasia

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PROXIMAL VARIANT -consists of large epithelioid OR rhabdoid cells has a more aggressive clinical course Also referred to as "large cell epithelioid sarcoma" due to presence of large rhabdoid cells Necrosis often appears in the center of nodules

Ultrastructure :

Ultrastructure Epithelioid cell exhibit abundant intermediate filaments

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S100 - negative (distinguishing it from malignant peripheral nerve sheath tumour) Endothelial markers- negative (distinguishing it from epithelioid angiosarcoma) CK5/6 -negative (distinguishing it from squamous cell carcinoma) P63 negative ( distinguishing it from squamous cell carcinoma)

Treatment :

Treatment Wide surgical resection remains the most recommended treatment modality. Epithelioid sarcoma has recurrence rates of up to 77% after marginal resection Amputation can be considered if there are multiple recurrences Adjuvant Chemotherapy with doxorubicin and radiation therapy

Prognosis :

Prognosis Five year survival and ten year survival rate for patients with epithelioid sarcoma are approximately 50-70% and 42-55% respectively Gender, site, age of diagnosis, tumor size and microscopic pathology

Challenges in Diagnosis:

Challenges in Diagnosis Slow growth of the tumor Paucity of symptoms Benign appearance in early stage Closely mimic various non- neoplastic lesions All these often evades timely diagnosis

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Fibrous Histiocytoma of Orbit : A Case Report MANJIT S BAL, SP SINGH, KRISHNA JINDAL, KUSUM K THAKUR Deptt . of Pathology, Govt. Medical College, Patiala . Indian journal of Pathologist and Microbiologists 43 (1) : 93- 95, 2000 Case 2

Clinical Presentation:

Clinical Presentation 50 y Male H/o Proptosis left eye since 1 year Pain since last 15 days Preoperative X-Ray and CT scan - erosion of roof of maxillary and floor of frontal sinuses Clinical Diagnosis – Malignant tumor of Left Eye Management - Enucleation of Left Eye along with mass and sent for histopathological examination.

Histopathology:

Histopathology Gross – specimen of eyeball with cut optic nerve, ocular muscles and mass Mass measured 5 X 3 X2.5 cm It was attached to posterior aspect of eye ball with a thin loose fascia Outer surface of mass was slightly nodular & firm in consistency Eye ball was normal in size Cut surface - eyeball revealed no abnormal findings Mass was homogenous grayish white in colour.

M/E:

M/E Presence of fibrous connective tissue capsule enclosing a spindle cell neoplasm Fibroblast like cells arranged in bundles and fascicles Storiform pattern was evident at many places Nuclei were round to ovoid and spindled 1 mitotic figure / 4 HPF Areas of myxoid change, many vascular spaces, few microcystic spaces, small number of lymphocytes & some extravasated RBC Final Diagnosis- Benign Mesenchymal tumor – Fibrous Histiocytoma ( Dermatofibroma )

Discussion :

Discussion FH of orbit typically occur in middle age Deeply situated FH tends to be larger than cutaneous ones i . e. 5cm or larger They have a more distinct storiform pattern and fewer secondary elements like xanthoma cells as compared to cutaneous FH

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Font and Hidayat (1982) divided orbital fibrous histiocytomas into benign, locally aggressive and malignant with 10 yr survivial of 100%, 92% and 23% respectively. FH has to be managed by exenteration since invasion of bone can lead to death from local causes even in the absence of metastasis.

Fibrohistiocytic Tumors:

Fibrohistiocytic Tumors Characterized by dual cellular composition cells with fibroblastic appearance intimately mixed with cells of morphologic and functional attributes of histiocytes

WHO 2002 classification:

WHO 2002 classification Benign Giant cell tumour of tendon sheath Diffuse-type giant cell tumour Deep Benign Fibrous Histiocytoma Intermediate (rarely metastasizing) Plexiform fibrohistiocytic tumour Giant cell tumour of soft tissues

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Malignant Pleomorphic ‘MFH’ / Undifferentiated pleomorphic sarcoma Giant cell ‘MFH’ / Undifferentiated pleomorphic sarcoma with giant cells Inflammatory ‘MFH’ / Undifferentiated pleomorphic sarcoma with prominent inflammation

Benign Fibrous Histiocytoma Cutaneous / Superficial:

Benign Fibrous Histiocytoma Cutaneous / Superficial Very common benign, indolent tumour of adults, common site is legs of women 20-50 years old May be associated with trauma Multiple lesions may be associated with immuno -suppression.

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Gross Description- Tan-brown, firm, mobile, painless papule < 2 cm in dermis Size varies slightly with time; rarely > 5 cm and plaque-like May dimple upon lateral compression; may have overlying scaling

M/E:

M/E Well defined but non-encapsulated Storiform pattern of spindled and bland fibroblasts and histiocyte -like cells in mid-dermis and subcutaneous tissue with infiltrative margins but sparing epidermis Foamy histiocytes with variable hemosiderin , some multinucleated giant cells, branching vessels, chronic inflammatory cells Pseudoepitheliomatous hyperplasia and epidermal hyperpigmentation

Cytology :

Cytology Loose cell clusters Ovoid, kidney shaped or spindled nuclei Bland Granular chromatin Abundant granular / vacuolated cytoplasm Indistinct cell borders Multinucleated histiocytic giant cells

Deep Benign Fibrous histiocytoma :

Deep Benign Fibrous histiocytoma Benign fibrous histiocytoma of subcutaneous tissue, deep soft tissue or parenchymal organs, with no dermal involvement Usually adult males > 25 years Head and neck and lower limb Gross- Well circumscribed with pseudo-capsule, typically > 4 cm, variable hemorrhage

M/E :

M/E Prominent storiform pattern of uniform spindle cells with ill defined eosinophilic cytoplasm Often hemangiopericytoma -like vasculature Scattered lymphocytes, and either multinucleated giant cells, osteoclastic giant cells or foam cells in 59% Less than 5 mitotic figures/10 HPF Stroma is myxoid or hyaline

Variants :

Variants Cellular FH - Fascicular or storiform Cells are plumper than DFSP, with eosinophilic cytoplasm and tapering nuclei Mean 3 mitotic figures/10 HPF

Angiomatoid FH:

Non-endothelial lined clefts or lakes containing blood Aneurysmal FH Angiomatoid FH Thick peudocapsule , lymphoid aggregates

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Atypical FH Enlarged Atypical cells Epithelioid FH Epithelioid cells in hyalinized stroma

Juvenile Xanthogranuloma:

Juvenile Xanthogranuloma Proliferative disorder of dendrocytes in skin

Treatment :

Treatment Benign fibrous histiocytoma is treated by wide surgical excision, with 5-10% of cases recurring locally. Deeper and larger lesions have a higher rate of recurrence. More aggressive forms , such as with marked cellularity , mitotic activity, focal necrosis, even atypical giant cells are associated with poorer outcome. Difficult to predict biological behaviour Extended follow-up is recommended after surgical removal

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