Perioperative management of mastocytosis.

PURPOSE: Patients with mastocytosis have an increased risk of anaphylaxis during surgical procedures with general anesthesia. Therefore, we reviewed the anesthesia course of a large cohort of patients with mastocytosis. METHODS: We retrospectively reviewed adult and pediatric patients with mastocytosis who underwent surgical procedures with general anesthesia at Mayo Clinic from January 1, 2000, through June 30, 2021. We also included any procedures with general anesthesia that occurred during the 3-year period preceding mastocytosis diagnosis and designated the patients who underwent these procedures as having an unknown diagnosis at the time of their surgical procedure. We analyzed whether patients received chronic antimediator treatment for mastocytosis and/or prophylactic medications before the procedures. We also determined whether medications indicative of mastocytosis-related adverse events were intraoperatively administered. RESULTS: We identified 113 patients who underwent 219 procedures during the study period; 25 procedures were performed before mastocytosis diagnosis. Of 194 procedures in patients with known mastocytosis, patients received chronic antimediator therapy and/or perioperative prophylactic medications for 178 (91.8%) procedures. Among these procedures, 10 were potentially complicated by mast cell activation, which was inferred from administration of inhaled albuterol (n = 3) or intravenous diphenhydramine (n = 8). In addition, there was only one case of intraoperative anaphylaxis which occurred in a patient who underwent anesthesia before mastocytosis diagnosis and therefore did not receive prophylaxis. CONCLUSION: Intraoperative anaphylaxis can be the first presenting sign of mastocytosis. Patients with mastocytosis who received chronic antimediator therapy and/or preoperative prophylactic medications had an uneventful surgical course.

Adjuvant medical therapy provides no therapeutic benefit in the treatment of dogs with low-grade mast cell tumours and early nodal metastasis undergoing surgery.

Lymph node (LN) metastasis is a negative prognostic factor in dogs with cutaneous mast cell tumours (cMCTs). While elective lymphadenectomy of metastatic LNs improves outcome, the benefit of adjuvant medical therapy in dogs with early metastatic (HN2) LNs is debated. The aim of this retrospective multicentre study was to evaluate the therapeutic benefit of adjuvant medical therapy following surgical removal of the primary low-grade cMCT (Patnaik grade 1-2 and Kiupel low-grade) and lymphadenectomy of HN2 LNs by analysing survival rates and patterns of recurrence. Seventy-three dogs were included: 42 received adjuvant medical treatment (chemotherapy and/or kinase inhibitors), and 31 did not. The median follow-up time for medically treated dogs was 619 days: two experienced local recurrence, three nodal relapse and four distant relapse. For dogs undergoing surgery only, the median follow-up time was 545 days. None of them experienced local recurrence, nodal, or distant relapse. Time to progression was significantly shorter in dogs receiving adjuvant medical treatment (P = .021). A similar tendency was observed for overall survival (P = .056). The current study shows that dogs with low-grade cMCTs, that undergo surgical excision of the primary tumour and elective lymphadenectomy of the HN2 regional LN harbour a good prognosis. The use of adjuvant medical treatment in these dogs does not seem to provide any benefit in terms of progression and survival.

A Rare Case of Spinal Mastocytosis Presenting as an Extradural Mass.

BACKGROUND: Systemic mastocytosis is a hematopoietic disorder of mast cell deposits in various systemic tissues that can include skin, spleen, liver, and bone marrow. When accumulation occurs within bone it has been reported to cause osteoporosis, osteosclerosis, or sometimes a mixed pattern. In exceptionally rare circumstances, it has been reported to present as an exophytic mass causing neural compression. CASE DESCRIPTION: A 72-year-old man was admitted with progressive worsening in his mobility and heavy and numb sensation in his bilateral lower limbs that worsened on walking. His weakness subsequently progressed such that he was no longer able to mobilize for 48 hours prior to admission. On examination he was found to be myelopathic with a sensory level noted at approximately the T10/11 area. Whole spine magnetic resonance imaging was conducted, which revealed a heterogeneous lesion involving the vertebral body and posterior elements of T10. The patient underwent T9-T11 laminecetomy with intralesional debulking of metastatic spinal column tumor. Postoperatively, the patient was noted to have improved anti-gravity power in the lower limbs. Intraoperative biopsy samples revealed focal paratrabecular infiltrates of abnormal mast cells with collagen fibrosis and focal bone destruction, along with cytogenetic features in keeping with systemic mastocytosis. CONCLUSIONS: We describe a rare case of systemic mastocytosis presenting as an extradural lesion causing compressive myelopathy and paraparesis. To our knowledge, this has only been described once before in literature. The patient underwent decompression surgery with improvement in neurology postoperatively. He was subsequently referred to the hematology team for further investigation and management.

Amount of skin shrinkage affecting tumor versus grossly normal marginal skin of dogs for cutaneous mast cell tumors excised with curative intent.

OBJECTIVE To assess differences in skin shrinkage between grossly visible tumor and grossly normal marginal skin of dogs for cutaneous mast cell tumors (MCTs) excised with curative intent and to determine an equation to estimate postexcisional gross tumor margins from preexcisional measurements and vice versa. SAMPLE 19 cytologically confirmed and surgically excised cutaneous MCTs obtained from dogs. PROCEDURES Tumors were measured in craniocaudal and dorsoventral directions before excision, immediately after excision, and after fixation in formalin. Both grossly visible tumor and surrounding grossly normal skin that comprised the surgical margin were measured at each time point. Percentage of shrinkage was compared among time points and between the tumor and surrounding grossly normal skin. Patient and histopathologic variables were correlated to skin shrinkage. RESULTS Overall shrinkage was 17.70%. The amount of shrinkage within the grossly visible tumor (4.45%) was less than that within the surrounding grossly normal skin (24.42%). Most of the shrinkage occurred immediately after excision. There was no effect of age, sex, completeness of excision, or degree of edema. Accuracy of an equation to estimate postexcisional margins from preexcisional measurements was only 18.4%. CONCLUSIONS AND CLINICAL RELEVANCE Grossly evident MCTs of dogs shrunk less than did the grossly normal surrounding skin. Although an equation to estimate postexcisional margins from preexcisional measurements could be derived, it likely would need to contain additional variables not included in the study reported here. Until such an equation exists, care must be used when extrapolating surgical margins from histologic margins and vice versa.

Inter- and intra-rater reliability and agreement in determining subcutaneous tumour margins in dogs.

The objective of this prospective study was to evaluate agreement and reliability of calliper-based measurements of locally invasive subcutaneous malignant tumours in dogs. Four raters measured the longest diameter of 12 subcutaneous tumours (7 soft tissue sarcomas and 5 mast cell tumours) from 11 client-owned dogs during 3 randomized, blinded measurement trials, both pre- and post-sedation. Inter- and intra-rater reliability was evaluated using intra-class correlation coefficient (ICC) and agreement was evaluated using Bland-Altman plots. Inter- and intra-rater reliability was good (ICC range of 0.8694-0.89520) and excellent (ICC range of 0.9720-0.9966), respectively. For agreement calculations, an a priori clinically relevant limit of agreement of 10 mm was set. Inter- and intra-rater agreement was unacceptable with inter-rater limits of agreement ranging from 15.9 to 55.6 mm and intra-rater limit of agreement ranging from 11.9 to 28.1 mm. Review of the measurement trial photographs revealed that calliper orientation changes were frequent, occurring in 9/12 (75%) and 8/12 (67%) pre- and post-sedation cases. No significant correlation was found between inter-rater measurement standard deviations and calliper orientation changes or dog body condition score. These findings suggest veterinarians may have poor agreement in determining the gross edge of tumours, which is expected to introduce bias and inconsistency in tumour staging, assessing response to therapy, and surgical margin planning. Due to the potential consequences for veterinary cancer patients, future studies are needed to validate the present findings.

Survey of UK-based veterinary surgeons' opinions on the use of surgery and chemotherapy in the treatment of canine high-grade mast cell tumour, splenic haemangiosarcoma and appendicular osteosarcoma.

The objective of this study was to determine the frequency of recommendation of adjuvant chemotherapy by UK-based general veterinary practitioners (GVPs) for canine patients with high-grade mast cell tumours (HGMCTs), splenic haemangiosarcomas (SHSs) and appendicular osteosarcomas (AOSs); to determine which chemotherapeutic protocols are used by GVPs; and to evaluate reasons why chemotherapy may or may not be recommended postoperatively. An internet survey was created in 2013 using an online programme and was distributed to GVPs. Questions relating to the use of adjuvant chemotherapy for three tumours were selected. In total, 300 responses were generated. Surgery was seen as primary therapy by most GVPs for HGMCT (91 per cent) and SHS (88 per cent), but less frequently for AOS (59 per cent). Also, 90, 40 and 57 per cent of respondents recommended adjuvant chemotherapy for HGMCT, SHS and AOS, respectively. Of these, an appropriate chemotherapy protocol was not known by 25, 51 and 36 per cent of GVPs for HGMCT, SHS and AOS, respectively. From the GVPs not recommending chemotherapy, 29, 64 and 66 per cent did not believe chemotherapy to be efficacious for these tumours. The frequency of recommendation regarding postoperative chemotherapy is variable by tumour type. Reasons given why postoperative chemotherapy is not recommended also vary by tumour type.

Current concepts in oncologic surgery in small animals.

Surgical oncology is experiencing rapid transition in veterinary medicine. Mast cell tumors and soft tissue sarcomas are two of the most common neoplasms in small animal patients. Clinicians should be familiar with the need for staging and the procedures involved in treating patients with these tumors. Clinicians should be comfortable with available adjuvant therapies and when to use them in certain patients.

Laparoscopic splenectomy: operative technique and outcome in three cats.

CLINICAL SUMMARY: The clinical findings, treatment and outcome for three cats that underwent laparoscopic splenectomy using bipolar vessel-sealing devices for resection of diffuse splenic disease are described. In each case, a three-portal laparoscopic technique was used. The spleen was manipulated and its mesentery and associated vessels sequentially cauterized and ligated to enable removal through a portal incision with minimal hemorrhage. Each of the three patients recovered from anesthesia without incident and was able to be discharged to the owner the next day. PRACTICAL SIGNIFICANCE: Laparoscopic splenectomy may be a safe and effective alternative to celiotomy in a select group of cats requiring splenectomy.

Cutaneous mast cell tumor and mastocytosis in a black-masked lovebird (Agapornis personata).

A 12-year-old female black-masked lovebird (Agapornis personata) with a cobalt color mutation was presented for self-mutilation of a mass located on the right lateral neck. Cytologic evaluation of the soft tissue mass revealed a predominance of poorly stained mast cells with metachromatic intracytoplasmic granules. The presumptive diagnosis was cutaneous mast cell tumor. Clinical evaluation, results of a complete blood cell count and biochemical analysis, and radiographs did not reveal systemic manifestation of mast cell disease. The mass was surgically resected, but surgical margins were limited because of the location of the mass and the small size of the patient. The lovebird died the day after surgery. Gross postmortem examination revealed splenomegaly, multifocal pinpoint white nodules throughout the liver parenchyma, severe thickening and yellow coloration of the great vessels, and pale pink swelling of the caudal right kidney. Histopathologic analysis of the resected mass revealed sheets of round cells that contain metachromatic granules, defined as neoplastic mast cells, within a fine fibrovascular stroma. Similar neoplastic cells were seen in the right kidney, hepatic sinusoids, splenic pulp, periovarian connective tissue, and bone marrow. The histopathologic diagnosis was a cutaneous mast cell tumor and disseminated mast cell disease, or mastocytosis. To the authors' knowledge, this is the first reported case of a cutaneous mast cell tumor and mastocytosis in a psittacine bird.

Mast cell tumour and cutaneous histiocytoma excision wound healing in general practice.

OBJECTIVE: To assess whether wounds from incomplete mast cell tumour excisions are at greater risk of healing complications than wounds from complete excisions, or cutaneous histiocytomas. METHODS: Mast cell tumours and cutaneous histiocytomas submitted to Nationwide Laboratories between November 1, 2007 and April 30, 2008 were selected. Questionnaires were sent to submitting veterinarians requesting details of tumour characteristics, clinical approach to the tumour and wound healing. RESULTS: Three hundred and eighty-six mast cell tumours and 524 cutaneous histiocytomas were identified. One hundred and eighty-five mast cell tumours and 244 cutaneous histiocytomas questionnaires were returned (47% response). Wound complications arose in 20% of mast cell tumours and 21% of cutaneous histiocytomas. Multivariable analysis confirmed that larger tumours, tumours on the feet and a soft/"baggy" appearance, were significantly associated with a greater frequency of problems, leading to delayed wound healing and dehiscence. CLINICAL SIGNIFICANCE: Incomplete mast cell tumour excision does not lead to greater risk of wound complications. Mast cell tumour surgical wounds have a similar rate of wound complications as cutaneous histiocytoma wounds.

Outcome following splenectomy in cats.

No studies have yet examined whether there are prognostic factors for survival for cats undergoing splenectomies. The medical records of 19 cats that had complete splenectomy were reviewed for information on preoperative, intraoperative, and postoperative factors. The most common presenting signs were a palpable abdominal mass in 58% and anorexia in 47% of the cats. Mast cell tumors were the most common reason for splenectomy and were found in 10/19 cats (53%); followed by hemangiosarcoma in 4/19 (21%); and lymphoma in 2/19 (11%). The Kaplan-Meier median survival time (MST) was 197 days, with a range from 2 days to 1959 days. Three cats were noted to have preoperative weight loss, and this was the only factor that had prognostic significance for survival following surgery. For cats with weight loss the MST was 3 days, for cats with no weight loss noted the MST was 293 days (P=0.008).

Mast cell tumours and other skin neoplasia in Danish dogs--data from the Danish Veterinary Cancer Registry.

BACKGROUND: The Danish Veterinary Cancer Registry (DVCR) was established in May 2005 to gather information about neoplasms in the Danish dog and cat populations. Practitioners from more than 60 clinics throughout Denmark have submitted data on these species. The objectives of the current study were, with a special focus on mast cell tumours (MCT) to investigate the occurrence, gender distribution, biological behaviour, locations, types, the diagnostic method used and treatment of skin neoplasms in dogs based on information reported to the DVCR. METHODS: From May 15th 2005 through February 29th 2008, reports on a total of 1,768 canine cases of neoplasia in the skin, subcutis or adnexa were submitted.) Of these, 765 cases (43%) were confirmed by cytology or histopathology. RESULTS: The majority of dogs had a benign neoplasm (66%) while 21% were cases of malignant neoplasia. The most commonly encountered malignant neoplasms were MCT and soft tissue sarcomas and for benign neoplasms, lipomas and histiocytomas were the most common. The location of the neoplasms were primarily in the cutis, subcutis or in the perianal region. The occurrence, gender distribution, biological behaviour and location of canine skin neoplasias in Denmark were similar to earlier reports, although some national variations occurred. A correlation between grade of MCT and the proportion of cases treated surgically was observed. CONCLUSIONS: Population based cancer registries like the DVCR are of importance in the collection of non-selected primary information about occurrence and distribution of neoplasms. The DVCR provides detailed information on cases of skin neoplasms in dogs and may serve as a platform for the study of sub-sets of neoplastic diseases (e.g. MCT) or subgroups of the canine population (e.g. a specific breed).

Mastocitosis en la práctica anestésica. A propósito de un caso / Mastocytosis and anesthesia: a case report

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[Mastocytosis: general anesthesia with remifentanil and sevoflurane]. / Mastocytose: anesthésie générale par rémifentanil et sévoflurane.

Mastocytosis is a disorder with potential anaesthesia complications. Reviewing the literature, anaesthetic management of mastocytosis is controversial. We report the successful use of remifentanil and sevoflurane in a woman with systemic mastocytosis. No reaction nor histamine release was observed in these cases.

A solitary manifestation of mastocytosis in the head and neck.

Solitary mastocytosis in adulthood is a rare finding. Only two such lesions have been reported in the head and neck. We describe a 27-year-old woman who had a 10-year history of a forehead swelling that had fluctuated in size. Light trauma or pressure on the lesion resulted in an increase in its size. A mass was found to be situated just below the galea and was successfully removed surgically using a high forehead lift. Histologically, the specimen contained predominantly mast cells. A systemic mastocytosis was excluded by a multidisciplinary diagnostic approach and measurement of the 24-h urinary excretion of histamine metabolites. After 36 months of follow-up there has been no recurrence.

Telangiectasia macularis eruptiva perstans: a case report and review of the literature.

We report a 20-year-old female patient with telangiectasia macularis eruptiva perstans characterised by telangiectatic macular lesions and episodic flushing, two lesions of which were successfully treated by laser. Two clinically different forms of cutaneous mastocytosis have both been previously described as telangiectasia macularis eruptiva perstans, and we now propose that this term be restricted to cutaneous mastocytosis characterised by the typical telangiectatic macules.

Case report of bone mastocytosis: total hip arthroplasty for osteoarthritis and open reduction for condylar fracture of the knee.

A 72-year-old woman was admitted in 1984 for painful protrusive osteoarthritis of the left hip diagnosed as systemic mastocytosis with bone lesions and clinical features of intestinal malabsorption but no clinical skin lesion. The total hip replacement, refused in a first step because of the bone pathology, was carried out two years later. Signs of loosening appeared after one year. In 1990, following a traumatic bicondylar fracture of the left knee, an osteosynthesis was carried out. Ten days later, a shaft pathologic fracture of the femur above the osteosynthesis plate implied another open reduction. Two and a half years later, the patient is able to walk short distances, using walking sticks, and lives at home receiving social assistance.