4.

CLINICOPATHOLOGICAL AND ORAL PATHOLOGICAL

CASE DEMONSTRATIONS
REPORT OF RARE DISEASES

Balázs Dezső, MD, PhD

UD Faculty of Medicine
Department of Pathology
2020
Dear Dentistry Students,

The following Clinicopathology case demonstrations, representing rare

diseases (i.e., the first two cases) are intended for a brief review by you,
only. These are equipped with diagnoses. Therefore, you do not need to
do anything with them – except reading and incorporating those pieces of
information which you feel important. When having the elective sessions
for consultation during the upcoming week, however, I will review those
with the related diseases (e.g., lymphomas, and some autoimmune
disorders) which are more common, and more importantly, needed to be
known by you for the final examination in Pathology.

Following these cases you have received seven common oral pathology
cases via images. I would appreciate if you could recognize at least the
nature of lesions by their gross appearances and provide just a diagnosis,
together with the anatomical location (if applicable) and in turn send your
answers in email to me (not in an attached files!!). As usual, the deadline
is Sunday, midnight.
Good luck!

Thanks for your co-operation.

Best regards
Balazs Dezso
 1. Eset ANAMNESZTIKUS ADATOK
43 éves nő
HISTORY OF PATIENT
Case 1 43 year female

• Korábban alkoholista, de 2 éve absztinens. Azóta pszichiátriai

kezelés alatt áll, szedatívumokat szed.
• Earlier she was an alcoholist but for two years she has been
abstinent. She has been ever since having psychiatric problem

• Ismert scleroderma, a DE OEC III.sz. Belklinikán gondozzák. From

her previous history: scleroderma which is being managed with
corticosteroids and followed up by the doctors of UD, Institute of
Internal Medicine.

• Egyéb lényeges betegsége korábban nem volt. No other significant

diseases were recorded for this patient.
 PANASZOK, KLINIKAI TÜNETEK
COMPLAINTS, CLINICAL SYMPTOMS

• Nyaki nyirokcsomó megnagyobbodás, bőrviszketés,

fáradékonyság.
• Lymph node enlargement on the neck

Sátoraljai kórházi kivizsgálás- Findings at the local

Hospital (Sátorújhely):
• Számos 1-2 cm-es nyirokcsomó a nyak mindkét oldalán,
valamint a bal hónaljárokban.
• Several palpable lymph nodes (1 to 2 cm) on both sides of the
neck
• Egyéb szervi eltérés nincs, máshol megnagyobbodott nyirokcsomó
nem detektálható, laboratóriumi paraméterek és a vérkép
physiológiásak. A vérsüllyedés azonban magas.
• No other remarkable finding. Laboratory chemistry is within normal
limits except high blood sedimentation.
 SÁTORALJAÚJHELYI KIVIZSGÁLÁS:
EXAMINATIONS:
• Az egyik nyaki nyirokcsomó diagnosztikus célú eltávolítása:
• Surgical removal of one of the enlarged lymph node for
histopathologic diagnosis:
Szövettani diagnózis - Histopathol diagnosis:
Anaplasticus B-sejtes diffúz non-Hodgin lymphoma (atypusos
CD30+ sejtek jelenléte alapján)
Anaplastic diffuse B-cell non-Hodkin Lymphoma (based on
CD30+ cells)

Terápiás protokoll felállítása érdekében a

nyirokcsomóból készült szövettani vizsgálati anyaggal
együtt a beteget a budapesti Országos Onkológiai
Intézetbe utalják. For therapy, the patient with the
histopathology slides (for second opinion) had been
sent to the NATIONAL ONCOLOGY CENTRE, Budapest.
 ORSZÁGOS ONKOLÓGIAI INTÉZET:
NATIONAL ONCOLOGY CENTRE:
• Onkopathológiai szövettani revízió:
A Sátoraljaújhelyen eltávolított megnagyobbodott
nyirokcsomó reaktív.
• The ONCOPATHOLOGIC revision:
The lymph node which was removed in Sátoraljaújhely is
reactive.
Revíziós diagnózis – REVISED DIAGNOSIS:
Dermatopathiás lymphadenopathia
Dermatopathic lymphadenopathy with no
malignancy.
A beteg Sátoraljaújhelyre visszautalva The patient was sent back to
Sátoraljaújhely.
 THE FATE OF THE PATIENT:
• Diffuse cutaneous erythematous itchy papulous lesions
developed with muscle weakness.
The lymph nodes showed further enlargements especially
in the axillary lymph nodes. Therefore, the patient was sent
to the Lymphoma Centre of the University of Debrecen.
Examinations in the Univesity of Debrecen:
There were multiple erythematous papules with psoriasiform
plaque-like lesions.
Immunological and hematological examinations
and other laboratory parameters did not reveal
significant changes.

New lymph node removal from the axilla + skin

biopsy to establish new diagnosis.

Assumed diagnosis by the clinician: Lymphoma?

Sezary lymphoma?
HISTOPATHOLÓGIA-HISTOPATHOLOGY
HÓNALJI NYIROKCSOMÓ
AXILLARY LYMPH NODE

HE, 1x
HISTOPATHOLÓGIA-HISTOPATHOLOGY
HÓNALJI NYIROKCSOMÓ
AXILLARY LYMPH NODE

HE, 40x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ-AXILLARY LYMPH NODE

CD3, 10x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ-AXILLARY LYMPH NODE

CD20, 10x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ-AXILLARY LYMPH NODE

CNA-42, 10x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ AXILLARY LYMPH NODE

CD30, 10x
IMMUN-HISTOPATHOLÓGIA- IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ AXILLARY LYMPH NODE

CD30, 40x
IMMUN-HISTOPATHOLÓGIA- IMMUNOHISTOCHEMISTRY
HÓNALJI NYIROKCSOMÓ AXILLARY LYMPH NODE

CD1a, 20x
Nyirokcsomó diagnózis:
Dermatopathiás lymphadenopathia.

Axillary lymph node diagnosis:

dermatopathic lymphadenopathy
HISTOPATHOLÓGIA-HISTOPATHOLOGY
FELKAR BŐR-skin biopsy from upper arm

HE, 10x
FELKAR BŐR
SKIN BIOPSY

HE, 40x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
FELKAR BŐR
SKIN BIOPSY

CD3, 10x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
FELKAR BŐR
SKIN BIOPSY

CD3, 40x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
FELKAR BŐR
SKIN BIOPSY

CD4, 10x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
FELKAR BŐR
SKIN BIOPSY

CD8, 4x
IMMUN-HISTOPATHOLÓGIA-IMMUNOHISTOCHEMISTRY
FELKAR BŐR
SKIN BIOPSY

CD8, 40x
MOLEKULÁRIS PATHOLÓGIA-MOLECULAR PATHOLOGY
BŐRBIOPSIÁBÓL
SKIN BIOPSY
TCR-gamma specifikus primerekkel végzett nested
PCR reakció monoklonalitást igazol
By using TCR-gamma specific primers for nested PCR reaction
this revealed a monoclonality
 SUMMARY

DIAGNOSIS:
PRIMARY CUTANEOUS CD8+ EPIDERMOTROP CYTOTOXIC T-
CELL LYMPHOMA
MICROSCOPY
• Atypical lymphocytes intraepidermal infiltration in a band
like fashion, with brisk mitoses.
• Subepidermal lymphocytic infiltrates with reactive
eosinophils and plasma cells.

IMMUNHISTOCHEMISTRY
• Intraepidermal CD8+ neoplastic lymphocytes.
• Subepidermal CD4+ reactive lymphocytes

MOLECULAR PATHOLOGY
• Microdissection from the epidermal neoplastic lymphoid
infiltrates:
TCRg gene-rearrangement in a monoclonal fashion=NEOPLASM.
Histopathological case demonstrations

Balazs Dezso, MD, PhD

Institute of Pathology and Department of Oral
Pathology, University of Debrecen MHSC and Faculty
of Dentistry, Hungary

Clinicopathol and oral pathol case demonstr, 2020

 Case 2
40 year old female

Case history:
• She has been treated with SLE, predominantly manifested in glomerular disease since
the age of 23.
• Her disease showed moderate to mild clinical and serological activity including
antiphospholipid antibody (ab) positivities in low concentration (anti-b2 ph-glycopro-
tein IgG, anti-prothrombin, cardiolipin) but no thrombotic clinical symptoms had been
observed.

➢ At admission she presented pulmonary embolism preceded by respiratory tract

infection. Parallel with this event the anti-thrombin ab levels increased significantly.
➢ Despite of the long-term anticoagulant therapy, 4 weeks later dramatic deterioration
occured in patient’s clinical status manifested in hemiparesis, aphasia and impaired
consciousness caused by cerebrovascular thrombosis. In addition, a multi-organ
failure developed including heart, pulmonary and renal ivolvement.
The clinical picture seemed as a catastrophic antiphospholipid syndrome, CAPS.
Pulmonary microembolisation – pulmonális mikroembolizáció
 Thrombus in the left ventricule (despite of the oral acenocoumarol therapy)
Trombus a bal szívkamrában (dacára az orálisan adott acenocoumarol terápiának)
occlusion in medial cerebral artery on the skull CT- art cerebri media okklúzió
Thrombosis in right external jugular vein on virtual CT angiography
A j. vena jugularis internában trombus a CT angiográfián
Ischaemic lesion in the kidney – ischaemiás elváltozások a vesében
Renal biopsy was taken to confirm the clinical diagnosis of catastrophic
anti-phospholipid syndrome (CAPS)
Vese biopsziát végeztek, hogy megerősítsék a klinikailag diagnosztizált
katasztrofális anti-foszfolipid szindromát (CAPS)
Chronic mesangioproliferative GN (glomerulonephritis)
 Mesangial IgM deposits (cryostat section)
Glomeruli
 A B

Anti-fibrin-FITC (fluorescein, green)

Vesebiopszia glomerulusokkal (nyilak)
Kidney biopsy with glomeruli (arrows)
Glomeruli with various staining method
to demostrate thrombotic/fibrin deposits
in the capillary tufts (arrows) –
Glomerulusok különböző festésekkel,
hogy a kapillárisokban megjelenő
trombust/fibrint demonstrálja (nyilak)

C D

AFOG Masson trichrome

 BM
A B
L SE

M L L

Elektromikroszkópos kép glomerulusról: bazalis membrán és a fal megvastagodott a

kapillárisban (A) és mezangiálisan elektron-denz depozitumok, ami Ig lerakódást jelez (A).
M: mesangium; L: Kapilláris lumen; BM: basalis membrán
Electron microscopy of the glomerulus: Note the vascular basement membrane thickening
(A) and mesangial elecrto-dense deposits indicating Ig deposition (B). A, 8000x; B, 18 000x
magnifications.
M: mesangium; L: capillary lumen; BM: basal membrane.
A

CD34-TMR+C9-FITC

CD3-Texas Red+
CD20-FITC
Glomerular immun-deposition mediated lupus nephritis (LN)
LN, Class IV, A/C

CPM

IgG

C3

Oil-R-O
 Glomerular immun-deposition-mediated lupus nephritis
LN, Class IV, A/C

CD31DAB CD31DAB
CD3VIP CD163VIP

CPM
treatment
Take home message:
•This is a rare life threatening complication of antiphospholipid
syndrome APS mainly associated with SLE and induced by
infection.

Criteria for the classification of catastrophic APS

1. Evidence of involvement of three or more organs, systems and/or tissues
2. Development of manifestations simultaneously or in less than a week
3. Confirmation by histopathology of small vessel occlusion in at least one organ
or tissue
4. Laboratory confirmation of the presence of antiphospholipid antibodies
(lupus anticoagulant and/or anticardiolipin antibodies)
ORAL PATHOLOGY CASES
 Case 1
9 year old boy

• A cyst has been removed from the maxillary premolar region as

a follicular cyst.

1. ESET
2. 9 ÉVES FIÚ

• Maxilláris praemoláris régióból lett egy cysta eltávolítva,

mint folliculáris cysta.
Case 1
Case 1

PAS
Case 1

CK Ki-67 Vimentin
 1. eset, dg - Case 1, diagnosis

• Hamartomatous odontogenic ameloblastic

cyst (ameloblastic fibro-odontoma)
• Hamartomatosus odontogén ameloblastos
cysta (ameloblastos fibro-odontoma)

Take home message:

• Ameloblastic fibro-odontoma maybe considered as
an odontogenic malformation. Ameloblastos fibro-
odontoma esetenként (pl. ha cystaként jelentkezik)
hamartogén elváltozásnak is tekinthető.
Case 2

Diagnosis?
Case
Dg: 2
lichen planus
Diagnosis?
Case 3

Diagnosis?
Case 4

Diagnosis?
Case 5

Diagnosis?
Case 6

Diagnosis?
Case 7

Diagnosis?