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CHAPTER 21  Mast Cell Tumors  387



            TABLE 21.5     World Health Organization Clinical    therapy (RT) achieved long-term survival. 124  Other studies have
                       Staging System for Mast Cell Tumors       shown that dogs with intermediately differentiated MCTs with
                                                                 LN metastasis may have a good prognosis if the affected LN is
  VetBooks.ir  Stage  Description                                removed and adjuvant chemotherapy and/or RT is adminis-
                                                                             For poorly differentiated tumors, the presence of
                                                                 tered.
                                                                     114,120,125
             0
                   One tumor incompletely excised from the dermis, identified
                     histologically, without regional lymph node involvement  LN metastatic disease resulted in an MST of 194 days compared
                                                                                                 84
                    a.   Without systemic signs                  with 503 days for dogs with no metastasis.  For these dogs, treat-
                    b.   With systemic signs                     ment of the LN improved MST (240 days) compared with those
                                                                                                    84
             I     One tumor confined to the dermis, without regional lymph   dogs whose LNs were not treated (42 days).  As with all cases,
                     node involvement                            clinical judgment regarding LN metastasis is probably important.
                    a.   Without systemic signs                    Several miscellaneous factors have been linked to prognosis in
                    b.   With systemic signs                     dogs with MCTs. Certain breeds of dogs such as boxers, pugs,
                                                                 and dogs of bulldog descent appear to develop MCTs that often
             II    One tumor confined to the dermis, with regional lymph node   behave in a more benign fashion. 1,17,57,126  Recent rapid growth has
                     involvement
                    a.   Without systemic signs                  been associated with a worse outcome. For example, in one study,
                    b.   With systemic signs                     83% of dogs with tumors present for longer than 28 weeks before
                                                                 surgery survived for at least 30 weeks compared with only 25%
             III   Multiple dermal tumors; large, infiltrating tumors with or   of dogs with tumors present for less than 28 weeks.  Systemic
                                                                                                          57
                     without regional lymph node involvement     signs of hyporexia, vomiting, melena, widespread erythema, and
                    a.   Without systemic signs                  edema associated with vasoactive substances from MC degranula-
                    b.   With systemic signs
                                                                 tion are more commonly associated with visceral forms of MCT
             IV    Any tumor with distant metastasis, including blood or bone    and, as such, carry a more guarded prognosis. 58,68,127  In recent ret-
                     marrow involvement                          rospective studies of visceral or disseminated MCT, STs were short
                                                                 and nearly all dogs with follow-up died of their disease. 69,70,128
                                                                 Local tumor ulceration, erythema, or pruritus has been associated
                                                                 with a worse prognosis in some studies. 58,120  Lastly, recurrence
           and perineal region; however, when preputial and scrotal regions   of MCTs after surgical excision has also been associated with a
           were specifically evaluated, they were indeed associated with   more guarded prognosis. 92,118,120,129  Thus appropriate aggressive
           worse outcomes. 111,112  Approximately 50% to 60% of dogs with   therapy at the time of first presentation, rather than at the time
           MCTs located in the muzzle present with regional LN metasta-  of recurrence, may improve the long-term prognosis in patients
           sis. 113,115  Interestingly, this does not necessarily indicate a worse   with MCTs. 
           long-term prognosis, as the MST for dogs with metastatic disease
           was 14 months. 113  MCTs that originate in the viscera (GI tract,   Diagnostic Technique and Workup
           liver, spleen) or bone marrow carry a grave prognosis. 67,68  Recent
           data indicate that MCTs arising in the subcutaneous tissues have   MCTs are initially diagnosed on the basis of fine-needle aspira-
           a favorable prognosis with extended STs and low rates of recur-  tion (FNA) cytology. Rowmanovsky’s or rapid hematologic-type
           rence and metastasis. In one study of 306 dogs with subcutaneous   stains used in most practices will suffice. MCs appear as small- to
           MCTs, metastasis occurred in 4% of dogs and 8% experienced   medium-sized round cells with abundant, small, uniform cyto-
           local  recurrence. 103   The estimated  2- and  5-year  survival  prob-  plasmic granules that stain purplish red (metachromatic) (see Figs.
           abilities were 92% and 86%, respectively. Decreased survival was   7.2 and 7.5 of Chapter 7). 1,130  A small percentage of MCTs have
           linked to MI >4, infiltrative growth pattern, and presence of mul-  granules that do not stain readily, giving them an epithelial or
           tinucleation. 103  Lastly, conjunctival MCTs were found to have a   macrophage-like appearance that has often been referred to as giv-
           good prognosis, with 15 of 32 dogs disease-free at a mean of 21.4   ing a “fried-egg” impression (Fig. 7.2, Chapter 7). In these cases,
           months postsurgery; no dogs in this study died of MCT-related   a Wright–Giemsa or toluidine blue stain will often reveal gran-
           disease. 116                                          ules; however, histologic assessment may ultimately be necessary.
             Clinical stage, represented in  Table 21.5, is also predictive   Highly anaplastic, agranular MCTs can sometimes be challenging
           of outcome. 32,57,104,110,117,118  There is controversy regarding the   to definitively diagnose by routine light microscopy. Immunohis-
           effect of multiple MCTs on prognosis and, as such, this part of   tochemical techniques have been applied in an attempt to differ-
           the staging scheme may not accurately correlate with outcome.   entiate these from other anaplastic round cell tumors. MCTs are
           Several  studies  indicate  that  there  is  no  difference  in  outcome   vimentin positive and the majority are tryptase and CD117 (KIT)
           between patients with a single cutaneous MCTs and those with   positive. 38,131–133  Other markers that could potentially be useful
           multiple MCTs, 58,114,119,120  whereas others have suggested an infe-  include chymase, MCP-1, and IL-8. 9,10
           rior outcome in dogs with multiple tumors. 102,121  It is uncertain   Historically, preoperative diagnostic tests have included a
           whether this phenomenon represents an atypical form of metasta-  minimum database (complete blood count [CBC], serum bio-
           sis or multiple, unrelated tumors arising independently, although   chemistry profile), a buffy coat smear to document peripheral
           one study demonstrated a clonal origin for two distant cutaneous   mastocytosis, cytologic assessment of regional LNs, abdominal
           tumors arising over years. 122  The effect of LN metastasis on prog-  ultrasound (US) with cytologic assessment of spleen or liver if
           nosis is also somewhat controversial. In two studies, the presence   warranted, thoracic radiographs, and a bone marrow aspirate.
           of MCs in the regional LNs was a negative prognostic factor for   It is now likely that an extensive workup is unnecessary for dogs
           disease-free interval (DFI) and survival 120,123 ; however, an addi-  with MCTs that do not exhibit the previously discussed nega-
           tional study revealed that dogs with intermediately differentiated   tive prognostic factors. Fig. 21.4 illustrates the diagnostic steps
           MCTs and LN metastasis treated with postoperative radiation   and the order in which they are pursued in the authors’ practice.
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