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CFT II > Exam 4 - Histiocytic Sarcoma > Flashcards

Exam 4 - Histiocytic Sarcoma Flashcards

1
Q

what are the 2 main disease forms seen with histiocytic tumors?

A
  1. reactive histiocytosis - NOT neoplastic (autoimmune inflammation driven by T cells) - cutaneous or systemic
  2. neoplastic histiocytosis - histiocytes are truly neoplastic & drive the disease - cutaneous histiocytoma (benign), solitary histiocytoma (likes the elbows), & disseminated (acts like lymphoma)
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2
Q

T/F: histiocytic cancer is rare, and only 1% of tumors affect lymphoid tissue

A

true

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3
Q

what are your dendritic cells?

A

langerhan’s, kupffer, tissue antigen presenting cells, & macrophages

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4
Q

what are the different paths that may be taken from the common progenitor cell that results in the different forms of histiocytic tumors?

A
  1. common progenitor cell -> myeloid stem cell CD 34+ -> CD1+ CD14- = langerhans/dendritic cell = HISTIOCYTOMA (benign)
  2. common progenitor cell -> myeloid stem cell CD 34+ -> CD1- CD14+ -> HISTIOCYTIC SARCOMA CD1+CD11c+MHCII+CD4-Thy-Ecad- = REACTIVE HISTIOCYTOSIS (like lymphoma)
  3. common progenitor cell -> monoblast CD34- -> blood monocyte -> macrophage -> low CD1 expression, CD11c-CD11d+MHCII+ = HEMOPHAGOCYTIC HISTIOCYTIC SARCOMA WORST PROGNOSIS
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5
Q

what is hemophagocytic histiocytic sarcoma?

A

the worst type of histiocytic sarcoma - macrophages eat everything in the body (including RBC) - most animals don’t survive past 30 days

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6
Q

what dog breeds are the usual suspects for reactive histiocytosis?

A

flat coated retrievers

bernese mountain dogs

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7
Q

what are the stats on bernese mountain dogs getting cancer?

A

92% chance of getting cancer in their life

average life span of 6 years

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8
Q

what breed is worse than golden retrievers when it comes to getting cancer?

A

bernese mountain dogs

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9
Q

what is the lesion shown? what signalment of animal is often affected by it? where are they often seen?

A

reactive cutaneous histiocytosis - benign, diffuse aggregation of histiocytes in the skin

signalment - young to middle aged dogs with a possible male predilection

locations - head, pinna, scrotum, nasal planum, & mucosa

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10
Q

how is cutaneous histiocytosis diagnosed? how is it staged?

A

diagnosed - biopsy (want 2-3 samples, normal & abnormal junction), FNA is often difficult to distinguish between inflammation & granulomas

staging done even if cutaneous is suspected because it could be a worse prognosis if it is the systemic form - baseline blood work, chest rads, & lymph node aspirates (sternal lymph nodes drains skin of ventral chest/abdomen or cranial mediastinal lymph nodes if draining head & neck)

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11
Q

T/F: it is rare to have systemic histiocytosis that doesn’t involve the skin

A

true

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12
Q

what therapy is used for cutaneous histiocytosis?

A

tetracycline/doxycycline + niacinamide + vitamin e (helps the skin heal faster) = help suppress t-cell function & drive down the immune response

if that doesn’t work, can try steroids, chemo (lomustine most intense), etc - radiation for local lesions for dogs that are mostly in remission

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13
Q

what is the prognosis for cutaneous histiocytosis?

A

fair to guarded - spontaneous regression is possible, but chronic relapses & drug failures are common

euthanasia is done due to poor QOL

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14
Q

what breeds are predisposed to getting systemic histiocytosis?

A

bernese mountain dogs, rottweilers, golden retrievers, flat coated retrievers, & irish wolf hounds

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15
Q

what is systemic histiocytosis?

A

non-neoplastic disease of proliferating lymphocytes (autoimmune disease)

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16
Q

what animals are predisposed to getting systemic histiocytosis?

A

male, middle aged dogs

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17
Q

what common locations do we see systemic histiocytosis manifest?

A

subcutis, lymph nodes, bone marrow, liver, spleen, scrotum, lung, ocular tissue, & mucus membranes

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18
Q

T/F: the clinical signs seen in patients with systemic histiocytosis reflect the organs that are involved

A

true

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19
Q

what clinical signs are associated with systemic histiocytosis?

A

signs often wax & wane

anorexia, depression, weight loss, harsh breathing, & PU/PD (hypercalcemia)

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20
Q

what is the etiology of systemic histiocytosis?

A

dysregulation of the immune system - helper t cells

infiltration of lymphocytes DOES NOT indicate spontaneous regression in this disease!!!

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21
Q

what organ is most likely to be affected in cutaneous histiocytosis?

A

the liver (maybe spleen)

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22
Q

is PU/PD seen in systemic histiocytosis due to hypercalcemia of malignancy? why?

A

NO!!!

monocytes produce vitamin D analogue which drives calcium up!

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23
Q

does infiltration of lymphocytes indicate spontaneous regression in systemic histiocytosis? what about in a cutaneous histiocytoma?

A

systemic - NO!!!!

cutaneous histiocytoma - YES

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24
Q

how is systemic histiocytosis diagnosed? how is it staged?

A

biopsy - well differentiated benign histiocytomas, multinucleated giant cells, deep lesions in the skin, interstitial dendritic cells instead of langerhan’s (more superficial cell type)

staging - same as cutaneous form

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25
Q

what therapy is used for systemic histiocytosis?

A

same as cutaneous - start with steroids + tetracycline/niacinamide combo

try leflunomide (arava) before cyclophosphamide

26
Q

what is the prognosis for systemic histiocytosis?

A

not great?

periods of response to therapy with recrudescence (relapse quickly)

overall survival times range from 18-24 months with regressive therapy

euthanasia is done due to drug resistance & chronic recrudescence

27
Q

what differentials should you consider for systemic histiocytosis?

A

granulomatous disease & cutaneous histiocytosis

28
Q

what lesion is this? what animals are affected by it?

A

cutaneous histiocytoma - benign skin lesion, always epidermal in origin, non-haired, pink, rubbery, & fleshy that is derived from langerhan’s cells

occurs most often in younger dogs

29
Q

what is seen on FNA of a cutaneous histiocytoma?

A

round cells with abundant clear cytoplasm, vacuoles, eccentrically located nucleus, & small lymphoctes

30
Q

how are cutaneous histiocytomas diagnosed? what about staging?

A

FNA cytology is all you need

staging is generally not necessary as these often regress on their own

31
Q

what may happen to cutaneous histiocytomas as they regress?

A

may become reddened/ulcerated (lymphocyte mediated regression) - why owner’s bring their pet in

32
Q

when may you remove a cutaneous histiocytoma?

A

can be removed with narrow margins if it is causing the patient any discomfort - like on the eye or between the toes

33
Q

what clinical signs are seen with the visceral form of histiocytic sarcomas? what is the prognosis?

A

general malaise, anorexia, weight loss, cough/dyspnea

worse prognosis than periarticular

34
Q

what clinical signs are seen with the periarticular form of histiocytic sarcomas? what is the prognosis?

A

pain, swelling - can be super subtle (not palpable)

loves the elbows!!!

35
Q

how are histiocytic sarcomas diagnosed?

A

FNA is all you need - usually super characteristic appearance

can do special stains for CD18 & CD11

36
Q

what differentials should you consider for histiocytic sarcomas?

A

other round cell tumors, undifferentiated sarcomas, poorly differentiated synovial cell sarcomas

37
Q

what staging is done for histiocytic sarcomas?

A

generally same as the others

may see hemophagic syndrome on bloodwork - neutropenia, anemia, thrombocytopenia)

ultrasound to look at the liver & spleen - target lesion!!!!

lymph node & bone marrow aspirates if indicated

38
Q

what is the preferred treatment for histiocytic sarcomas?

A

surgery if possible!!! remove the primary lesion (don’t do it if there is already disseminated disease) - splenectomy, amputation, lung lobectomy

> 90% metastatic rate!!!

39
Q

what chemo is used for histiocytic sarcomas?

A

lomustine every 21 days as many times as needed to control disease - this is the most immunosuppressive therapy!!!

40
Q

what are the risks of treating a patient’s histiocytic sarcoma with lomustine?

A

this drug will drop platelets into the spontaneous bleeding range (like 35,000) & neutrophils to about 0

will cause hepatic fibrosis - give denamarin concurrently!!!

41
Q

what is the prognosis for histiocytic sarcoma?

A

poor!!!!

aggressive treatment with surgery & chemo MST 6 months

periarticular lesions or completely excised lesions maybe get 9 months

42
Q

what clinical signs are associated with disseminated histiocytic sarcoma? what organs are typically involved?

A

depression, anorexia, weight loss, vomiting, diarrhea, respiratory distress, jaundice - may have acute onset with rapid deterioration (cough to oxygen dependent & dying within a few weeks)

liver, spleen, lungs, lymph nodes, bone marrow, & skin

43
Q

how is disseminated histiocytic sarcoma diagnosed?

A

FNA, biopsy, special stains

CADET histiocytic sarcoma test (like BRAF) - on a cytology or biopsy sample (sensitivity 78% specificity 95%)

44
Q

when is a bone marrow aspirate indicated for disseminated histiocytic sarcoma?

A

only done if 1 lesion is seen - if more than 1 lesion, we assume it is everywhere

45
Q

why no steroids for disseminated histiocytic sarcoma?

A

shortens survival time

46
Q

what therapy is used for treating disseminated histiocytic sarcoma?

A

chemo - same as histiocytic sarcoma, lomustine every 21 days, l-doxorubicin every 12-21 days

47
Q

what is a negative prognostic factor for patients with disseminated histiocytic sarcoma?

A

thrombocytopenia is bad

48
Q

what is the prognosis for disseminated histiocytic sarcoma?

A

lomustine is the best drug we have & about 50% of cases actually respond…median survival time of 106 days & average remission time of 2 months

49
Q

what is hemophagic syndrome? what clinical signs are seen?

A

disseminated histiocytic sarcoma of macrophages where they eat all of the RBC (like 20 RBC inside 1 macrophage)

severe anemia (like 7%), jaundice, weakness/depression, anorexia, & collapse

50
Q

T/F: you should give blood to a dog that presents with hemophagic syndrome

A

false - it will just eat up the blood

51
Q

what organ is always involved if a dog has hemophagic syndrome?

A

the spleen

52
Q

what is seen on FNA that is supportive of hemophagic syndrome?

A

many criteria of malignancy are met + extreme phagocytosis

53
Q

what is the therapy used for hemophagic syndrome? what is the prognosis?

A

lomustine - really only 1 dose

extremely grave prognosis - only days

54
Q

T/F: feline histiocytic sarcoma is rare but when apparent is often disseminated in the spleen

A

true

55
Q

what organs are affected by histiocytic sarcomas in cats?

A

CNS, spleen, liver, lymph nodes, mediastinum, kidney, bladder, & bone marrow

very aggressive

56
Q

what therapy is used for histiocytic sarcomas in cats? what is the prognosis?

A

lomustine + l-doxorubicin

super poor prognosis!!!!

57
Q

what is malignant fibrous histiocytoma?

A

histologic description for a tumor consisting of malignant fibroblasts & histiocytes that are poorly differentiated from soft tissue sarcomas (more aggressive course)

58
Q

T/F: malignant fibrous histiocytomas are of histiocytic origin

A

FALSE - not of histiocytic origin

59
Q

how are dogs & cats affected by malignant fibrous histiocytomas?

A

dogs - subcutis & spleen

cats - feline injection site sarcomas

60
Q

what do you think this FNA sample came from?

A

cutaneous histiocytoma

61
Q

what do you think this FNA sample came from?

A

histiocytic sarcoma

62
Q

this lesion seen on ultrasound of the liver is associated with what disease?

A

histiocytic sarcoma!!!

CFT II (28 decks)

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