Case Presentation Maha Akkawi, MD, Fatima Obeidat, MD, Tariq - - PowerPoint PPT Presentation

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Case Presentation Maha Akkawi, MD, Fatima Obeidat, MD, Tariq Aladily, MD Department of Pathology Jordan University Hospital Amman, Jordan The 25th Annual Congress of the ADIAP The 8/11/2013 1 5th International Conference of JSP Clinical

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Case Presentation

Maha Akkawi, MD, Fatima Obeidat, MD, Tariq Aladily, MD Department of Pathology Jordan University Hospital Amman, Jordan

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 1

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Clinical Background

  • 39 year old smoker lady, presented with

a 6 month history of toothache and headache

  • Medical history: hypertension

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 2

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Clinical Background

  • Multiple visits to dental clinics, received

pain killers but without relief

  • Visited an ENT clinic, physical exam

revealed a nasal cavity mass

  • CT scan was performed

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 3

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CT scan

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Radiologic findings

  • Large, lobulated, destructive solid tumor

measuring 5 cm in maximum dimension, involving the roof of the mouth, the right nasal cavity, maxillary sinus, orbit

  • The tumor also extends to the right

infratemporal fossa, ptregopalatine fossa and parapharyngeal space, sparing the oropharynx

  • The patient underwent endoscopic biopsy

from the nasal mass

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 5

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Differential Diagnosis

  • Sarcoamtoid carcinoma(CK??)
  • Extracranial meningeoma (EMA, vimentin??)
  • Leiomyosarcoma ( Desmin, SMA??)
  • Solitary fibrous tumor (Bcl2, EMA??)
  • Anaplastic large cell lymphoma(CD30,CD45??)
  • Melanoma (HMB45??)

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 13

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cytokeratin

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vimentin

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CD45

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SMA

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Desmin

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S100

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EMA

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Bcl2

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CD30

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CD21

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CD23

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CD68

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Immunoprofile

Positive

  • vimentin
  • CD21
  • CD23
  • CD68 (focal)

Negative

  • S100
  • CD45
  • CK
  • EMA
  • SMA
  • Desmin
  • HMB45
  • CD99
  • Bcl2
  • CD20
  • CD30
  • ALK
  • CD1a

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 26

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Diagnosis

Extranodal follicular dendritic cell sarcoma

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 27

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FDC sarcoma

  • A rare neoplastic proliferation of FDCs

(antigen-presenting immune accessory cells that are widely distributed in tissues)

  • First reported by Monda et al, in 1986
  • Almost all patients are adults, with a median

age of 40 years and with no sex predilection

  • Some association with Castleman disease

(hyaline vascular variant)

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 28

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Etiology

  • Most cases: unknown
  • Hyaline-vascular Castleman disease (10-20%) of

cases

  • A pathway for tumor evolution: FDC hyperplasia,

dysplasia then transformation

  • Overexpression of p53 protein is noted in FDC

sarcoma, and is weakly positive in spindle cells in Castleman disease

  • Epidermal growth factor receptor expression

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 29

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Clinical symptoms

  • Patients often present with a slow-growing,

painless mass

  • Constitutional symptoms and paraneoplastic

syndromes are rare, unless associated with Castleman disease, or in inflammatory pseudotumor variant of FDC sarcoma

  • Abdominal tumors present with pain and

compression symptoms

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 30

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Nodal vs Extranodal sites

  • Nodal FDC sarcoma accounts for more than

two thirds of cases, with cervical lymph nodes being the most commonly affected

  • Extranodal FDC sarcoma is very rare (37 cases

by 2010)*

  • Most of the prototype: oropharynx
  • IPT-variant: spleen or liver
  • Other reported sites: GIT, soft tissue, skin, lung

and breast

* Duan GJ, Wu F, Zhu J, Guo DY, Zhang R, Shen LL, Wang SH, Li Q, Xiao HL, Mou JH, Yan XC, Extranodal follicular dendritic cell sarcoma of the pharyngeal region: a potential diagnostic pitfall, with literature review, Am j clin pathol, 2010 jan. 8/11/2013 31

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Gross features

  • Size range from 1 to 20 cm maximum

diameter, with a median size of 6 cm

  • Round to ovoid, well-circumscribed, fleshy

masses that on cut section are solid and tan

  • Interspersed areas of hemorrhage and

yellowish necrotic areas might be present particularly in larger tumors

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 32

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Microscopic features

  • The tumor is characterized by a proliferation of

spindle to ovoid cells that form fascicles, storiform patterns and whorls (360o)

  • Cells tend to be plump, with slightly eosinophilic,

fibrillary cytoplasm and indistinct cell borders

  • The nuclei are elongated, unevenly spaced, small

distinct nucleoli

  • Occasional nuclear pseudoinclusions

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 33

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Microscopic features

  • Small lymphocytes are scattered throughout

the tumor and perivascular cuffing

  • Occasional binucleated and multinucleated

tumor cells may be present

  • Although highly cellular, atypia is only mild to

moderate

  • Mitotic figures: 0-10/ 10HPF
  • Necrosis can be seen in higher grade tumors

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 34

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IPT variant

  • Spleen or liver
  • Prominent lymphoplasmacytic infiltrate,

masking neoplastic cells

  • Atypia is more variable within the tumor
  • Necrosis and hemorrhage are more common
  • EBV positive (EBER-ISH)
  • More aggressive

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 35

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Immunophenotype

  • Normal FDC antigens: CD21, CD35, CD23, D2-40,

CXCL-13

  • Common positivity: vimentin, fascin,

desmoplakin, HLA-DR, EGFR, CD4

  • Variable positivity: CD68, S100, EMA, CD15
  • Negative: CD45, CD20, CD1a, lysozyme, MPO,

CD34, CD3, CD79a, CD30, HMB-45, CK

  • Clusterin: sensitive and specific
  • Ki-67 index: 1-25%

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 36

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Treatment

  • The current approach is to apply therapeutic

guidelines similar to those used for soft tissue sarcomas of high grade

  • Complete surgical resection is the therapy of

choice

  • Adjuvant radiation or chemotherapy

decreases the rate of recurrence

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 37

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Biologic behavior and Prognosis

  • The behavior of these tumors is more akin to that of a

low-grade soft tissue sarcoma than a malignant lymphoma

  • Local recurrences rate: 36%, metastasis: 28%, mostly

to LN and liver Poor prognostic factors:

  • intra-abdominal location
  • size ≥ 6 cm
  • mitotic count ≥ 5 /10 HPFs
  • coagulative necrosis
  • significant nuclear pleomorphism

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 38

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Message?

  • As spindle cell tumors within lymph nodes are

rare, the diagnosis of FDCS within a node is not that difficult. However, extranodal FDCS cases are much more challenging in diagnosis because sarcomatoid carcinoma and sarcoma are by far much more common

  • Clue: morphologic features

The 25th Annual Congress of the ADIAP The 5th International Conference of JSP 8/11/2013 39

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Our patient

  • Patient still alive with disease
  • Receiving radiotherapy...
  • No surgery yet….

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