IARC Press Research Articles

ED07.02 The 2015 WHO Classification of Neuroendocrine Tumors

ED07.02 The 2015 WHO Classification of Neuroendocrine Tumors

Dysplastic Changes and Proliferation Index in Acquired Aplastic Anemia Are Not Associated with Progression to MDS/AML - a Study of Bone Marrow in Children and Adults

Acquired Aplastic Anemia (AAA) is a rare disease which progresses to MDS / AML in up to 15% of cases. When this happens, hematopathologists are asked whether the diagnosis of hypocellular Myelodisplastic Syndrome (h-MDS) would not have been confused morphologically with aplastic anemia.This study aims to identify morphological/immunophenotypical (M/I) findings that could predict this adverse prognosis in adults and children (<19y) diagnosed as AAA and verify if those criteria match with the ones described in literature in adult h-MDS (Bennett JM, Orazi A. Diagnostic criteria to distinguish hypocellular acute myeloid leukemia from hypocellular myelodysplastic syndromes and aplastic anemia: recommendations for a standardized approach. Haematologica, 2009;94:264-9)¹ and, more recently, in pediatric MDS (Baumann I, Niemeyer CM, Bennett JM, et al. Childhood Myelodisplastic Syndrome. In: Swerdlow SH, Campo E, Harris NL, et al (Ed.). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Fourth Edition. Lyon: IARC Press, 2008:104-7)², contributing to the discussion of this "grey zone".We retrospectively analyzed 123 patients/bone marrow (BM) biopsies at the moment of AAA diagnosis at Clinical Hospital of São Paulo Medical School from 1993 to 2012. Diagnosis of AAA was carried out according to classical criteria (Marsh JCW. et al. Guidelines for the diagnosis and management of aplastic anaemia. British Journal of Haematology, 2009;147:43-70). Evolution to MDS or AML was considered in the presence of at least one of the findings: significant dysgranulopoiesis or dysmegakaryocytopoiesis, more than 15% ring sideroblasts, blasts in peripheral blood or more than 5% blasts in bone marrow smear and/or biopsy, or in the presence of monosomy or deletion of the long arm of chromosome 7 by cytogenetic analysis (FISH or karyotype) of the BM. All biopsies were submitted to morphological (see Table 1) and immunophenotypic (MPO, Glycophorin A, Factor VIII, CD34, CD117 and Ki-67) evaluation by two hematopathologists without previous knowledge nor about the evolution of the patients neither cytogenetic analysis results. Nominal qualitative variables were analyzed by using Fisher’s exact test to check significant disproportion between the groups. The ordinal qualitative variables were analyzed for differences between groups by Mann-Whitney test. The significance level was 5% (a = 0.05). The correlation between the overall cellularity values of the samples and their proliferative index was evaluated by nonparametric Spearman r test.Seventy three (59%) were male, median age 25,3 years (7 months to 76years), 42 belongs to the pediatric group and 81 to the adults group. Median follow-up was 5,1y (range, 1 month to 22,1 years). Twelve patients (9,7%) (6 in each group) progressed to MDS/AML.Evaluation of M/I parameters are presented in Table 1. Criteria described by Bennett and Orazi¹ suggestive of h-SMD evaluated in our study were marrow dysgranulopoiesis, marrow dysplasia of megakaryocytes and CD34-positive blast cells in the presence of at least one of the previous two. Criteria described by Baumann et al.² suggestive of childhood MDS evaluated in our study were marrow dysgranulopoiesis, clusters of at least 20 erythroid precursors (1), increased number of proerythroblasts (2), increased number of mitoses of the erythroid elements (3) (criteria counted if 1+2, 1+3 or 2+3 were observed) and marrow dysplasia of megakaryocytes.There was no statistically difference in M/I findings as well as the presence of Bennett and Orazi¹ criteria for MDS and Baumann et al.² criteria for childhood MDS among total population, adults and children who developed and did not develop MDS/AML. There was a statistically significant correlation between the overall cellularity values of the samples and their proliferative index.Adult and pediatric patients with AAA, including those that progress to MDS/AML, have similar M/I characteristics. Some changes described by Baumann for pediatric MDS are also found in pediatric and adults cases with AAA. In addition, the proliferative index may be increased in cases of AAA and this finding has no correlation with progression to MDS/AML. M/I changes in bone marrow biopsies in AAA have failed to identify a group at higher risk for progression to MDS/AML in our series. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Azacitidine in Acute Myeloid Leukemia: Comparison of Patients with AML-MRF Vs AML-NOS Enrolled in the Austrian Azacitidine Registry

Azacitidine in Acute Myeloid Leukemia: Comparison of Patients with AML-MRF Vs AML-NOS Enrolled in the Austrian Azacitidine Registry

Myelodysplastic syndromes: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

P. Fenaux1, D. Haase2, G. F. Sanz3, V. Santini4 & C. Buske5 on behalf of the ESMO Guidelines Working Group* Service d’Hematologie Clinique, Groupe Francophone des Myelodysplasies (GFM), Hopital St Louis (Assistance Publique, Hopitaux de Paris) and Paris 7 University, Paris, France; Clinics of Hematology and Medical Oncology, University Medicine, Goettingen, Germany; Department of Haematology, Hospital Universitario y Politecnico La Fe, Valencia, Spain; Functional Unit of Haematology, AOU Careggi, University of Florence, Firenze, Italy; Comprehensive Cancer Center Ulm, Institute of Experimental Cancer Research, University Hospital, Ulm, Germany

Bladder cancer: ESMO Practice Guidelines for diagnosis, treatment and follow-up

Bladder cancer: ESMO Practice Guidelines for diagnosis, treatment and follow-up

Familial hypertryptasemia with associated mast cell activation syndrome

Familial hypertryptasemia with associated mast cell activation syndrome

Recently Described Sinonasal Tract Lesions/Neoplasms: Considerations for the New World Health Organization Book

Since the publication of the World Health Organization Classification of Head and Neck Tumors (Barnes et al., World Health Organization classification of tumours. Pathology and genetics head and neck tumours, IARC Press, Lyon, pp 10-80, 2001), a number of sinonasal lesions have been more completely described. This manuscript will focus on three such "new" lesions including sero mucinous hamartoma, HPV-related carcinoma with adenoid cystic-like features and low-grade sinonasal sarcoma with neural and myogenic features.

Primary Effusion Lymphoma: Secretome Analysis Reveals Novel Candidate Biomarkers with Potential Pathogenetic Significance

Primary Effusion Lymphoma: Secretome Analysis Reveals Novel Candidate Biomarkers with Potential Pathogenetic Significance

Unusual Presentation of Lymphoepithelioma-Like Carcinoma of Lung as a Thin-Walled Cavity

Most thin-walled cavities in the lung are benign lesions, but a few cases of lung cancer can have this unusual pattern. All previously reported cases were adenocarcinomas. Here we report a case of lymphoepithelioma-like carcinoma (LELC) of the lung presenting as a thin-walled cavity with a smooth inner surface. LELC is more commonly seen in Southeast Asia, including Taiwan, and its gross picture is usually a solid mass with a round, circumscribed border, which is indistinguishable from other non-small cell lung cancers. Asymmetric thickness of the cystic wall and lymphadenopathy are important features in the diagnosis and the selection of treatment.

Primary breast cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Primary breast cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Mimicry of sugar tumor and minute pulmonary meningothelial-like nodule to metastatic lung deposits in a patient with rectal adenocarcinoma

Several reports have described different lung lesions mimicking primary or metastatic neoplasms. In this paper, we describe the different features of two uncommon and benign lung lesions mimicking metastasis from a primary large bowel adenocarcinoma. Our patient is a 75-year old female with a history of invasive rectal adenocarcinoma. One month after her surgery, she started complaining of coughing and shortness of breath. Clear cell sugar tumor and minute meningothelial-like nodules had been found incidentally and simultaneously during her chest x-ray. The diagnosis had been made based on morphology and was supported by a positive staining to a panel of immunohistochemical stains including CD34, vimentin, HMB45, melan A and S100. An ultra-structural examination was also performed and confirmed the presence of melanosomes in sugar tumor. The coexistence of lung sugar tumor and minute pulmonary meningothelial-like nodules has never been reported in the literature and an awareness of these lesions is essential to correctly diagnose and stage patients.

Establishment and characterization of METON myoepithelioma cell line derived from human palatal myoepithelioma: apical reference to the diverse differentiation potential

Myoepithelioma is an extremely rare condition that accounts for 1–1.5 % of salivary gland tumors. It was formerly regarded as a subtype of pleomorphic adenoma, in which myoepithelial structural components predominated, but was listed as a separate disease entity in the 1991 World Health Organization classification (Seifert in Histological typing of salivary gland tumours. Springer, Berlin, 1991). Its histology is highly varied and recurrence is frequent (El-Naggar et al. in J Larygol Otol 103:1192–1197, 1989), with cases of malignant transformation having been reported (Seifert in Histological typing of salivary gland tumours. Springer, Berlin, 1991; Barnes et al. in Pathology and Genetics of head and neck tumours. IARC Press, Lyon, 2005), making this a difficult tumor to control in many cases. This is thought to be due to the multiple differentiation potential of myoepithelial cells, but the details are unknown. There have been a number of reports of the establishment of cell lines (Shirasuna et al. Cancer. 45:297–305, 1980; Jaeger et al. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 84:663–667, 1997), but numerous points remain unclear. We established a myoepithelial cell line designated METON, and investigated its characteristics. METON consists of cells with two different morphologies: spindle-shaped cells and epithelial-like cells. Then. we also used single-cell cloning method to establish various subclones (epithelial-like, spindle-like, and mixed epithelial-like/spindle-like cell lines). Among these, pluripotency markers were expressed by the mixed epithelial-like/spindle-like cell lines. The newly established cell line expressing these pluripotency markers will be extremely useful for elucidating the diverse histologies of salivary gland tumors.

R132C IDH1 Mutations Are Found in Spindle Cell Hemangiomas and Not in Other Vascular Tumors or Malformations

Spindle cell hemangioma (SCH) is a rare, benign vascular tumor of the dermis and subcutis. The lesions can be multifocal and are overrepresented in Maffucci syndrome, in which patients also have multiple enchondromas. Somatic mosaic R132C IDH1 hotspot mutations were recently identified in Maffucci syndrome. We evaluated the presence of mutations in solitary and multiple SCHs in patients without multiple enchondromas and tested a range of other vascular lesions that enter into the differential diagnosis. The R132C IDH1 mutation was identified by hydrolysis probes assay and confirmed by Sanger sequencing in 18 of 28 (64%) SCHs; of the 10 negative cases, 2 harbored a mutation in IDH2 (R172T and R172M) by Sanger sequencing. None of 154 other vascular malformations and tumors harbored an IDH1 R132C mutation, and R132H IDH1 mutations were absent in all 182 cases. All 16 SCHs examined by immunohistochemistry were negative for expression of HIF-1α. In conclusion, 20 of 28 (71%) SCHs harbored mutations in exon 4 of IDH1 or IDH2. Given that mutations were absent in 154 other vascular lesions, the mutation seems to be highly specific for SCH. The mutation does not induce expression of HIF-1α in SCH, and therefore the exact mechanism by which mutations in IDH1 or IDH2 lead to vascular tumorigenesis remains to be established.

Nasopharyngeal cancer: EHNS–ESMO–ESTRO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Nasopharyngeal cancer: EHNS–ESMO–ESTRO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Rare Primary Tracheal Tumor in a Middle-Aged Asian Male

Abstract SESSION TYPE: Cancer Cases IPRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PMINTRODUCTION: Ewing Sarcoma (ES)/Primitive neuro-ectodermal tumor (PNET) is a rare malignant tumor with a predilection for males with the ratio of 1.4 to 1[1]. This tumor usually occurs in bones and extraskeletal occurrences (particularly airways) is exceedingly rare[2].CASE PRESENTATION: A 45 year-old Asian non-smoking male with past medical history significant for coronary artery bypass on aspirin, plavix, and coumadin presented to the ER complaining of multiple episodes of hemoptysis over 24 hours. The patient was found to have coumadin-induced coagulopathy which was reversed with fresh frozen plasma. CT scan of the neck and thorax revealed only a mass in the proximal trachea. Bronchoscopy confirmed a tracheal mass on the posterior wall 3 cm from the carina. A 10mm x 9mm multi-lobulated mass was excised with electrocautery hot loop. H aE stained sections revealed hyperchromatic population of cells underlying the normal respiratory epithelium with moderate pleomorphism and mitotic activity. Immunoperoxidase stains were positive for vimentin and synaptophysin. They were negative for S100. Tumor was found strongly positive for CD99. Fluorescescence in-situ hybridization (FISH) demonstrated 99% of cells positive for EVSR1 gene at chromosome 22q12. Patient was given a final diagnosis of Ewing Sarcoma.DISCUSSION: ES /PNET is a malignant tumor that usually occurs in flat and long bones primarily in caucasian children and young adults rarely affects other ethnic groups [3]. Immuno-histochemical staining with CD99 is supportive of ES/PNET tumors is required to make a definitive diagnosis [2]. FISH procedure confirms the diagnosis through detection of the highly specific translocation of EWSR1 gene on 22q12 [3]. There have been reports of primary extraskeletal ES/PNET tumors in the lung, but only one has been reported in the tracheobronchial tree [1].CONCLUSIONS: The first case of primary ES/PNET of the trachea was described in 2010 by Elmi et al [1], which presented with acute airway obstruction and hemoptysis. We present the second case of ES/PNET in the trachea. However this is the first documented case on a middle age asian-male.1) Elmi, Maryam et al. Primary tracheal Ewing's sarcoma. Annals of Thoracic Surgery. 2010 oct;90(4):1349-52.2) Ushigome S, Machinami R, Sorensen PH. Ewing sarcoma/primitive neuroectodermal tumour (PNET). In: FletcherCDM, UnniKK, MertensF, editors. Pathology and genetics of tumours of soft tissue and bone. World Health Organization classification of tumours. Lyon: IARC Press, 2002: 297-3003) Jennifer Worch, et al.Racial differences in the incidence of mesenchymal tumors associated with EWSR1 translocation. Cancer Epidemiology, Biomarkers and Prevention. 2011 Mar;20(3):449-53DISCLOSURE: The following authors have nothing to disclose: Nelson Medina, Navneet Arora, Pratick Patel, Nazly ShariatiNo Product/Research Disclosure InformationNewark Beth Israel Medical Center, New Jersey, Iselin, NJ.

A “Twist box” Code of p53 Inactivation: Twist box:p53 Interaction Promotes p53 Degradation

Twist proteins have been shown to contribute to cancer development and progression by impinging on different regulatory pathways, but their mechanism of action is poorly defined. By investigating the role of Twist in sarcomas, we found that Twist1 acts as a mechanism alternative to TP53 mutation and MDM2 overexpression to inactivate p53 in mesenchymal tumors. We provide evidence that Twist1 binds p53 C terminus through the Twist box. This interaction hinders key posttranslational modifications of p53 and facilitates itsMDM2-mediated degradation. Our study suggests the existence of a Twist box code of p53 inactivation and provides the proof of principle that targeting the Twist box:p53 interaction might offer additional avenues for cancer treatment.

Somatostain receptor (SSTR)-targeted therapy in pulmonary large-cell neuroendocrine carcinoma (LCNEC) patients with intermediate proliferation rate (PR).

e17509 Background: LCNEC is classified as a high-grade (HG) foregut-derived neuroendocrine tumor (NET), exceeded only by small-cell lung carcinoma (SCLC) in terms of aggressiveness. It is a recent disease entity, only two decades old, and uncommon: 2%-3% of all lung cancer cases. Therefore, therapy guidelines are still evolving. Methods: A single institution observational study, consecutive series. Histopathology diagnosis conformed to WHO criteria for thoracic tumors (Travis et al, IARC Press, 2004). Immunohistochemistry included proliferation Ki67 index evaluation. SSTRs' affinity was assessed either by 111 In octreotate SPECT, or by Ga68 DOTA-NOC octreotate PET CT total body scans. Results: We have treated nine metastatic LCNEC patients over the last four years. Seven were scanned for presence of somatostatin receptors; 4 were positive. Three of the four had Ki67 staining ranging 10-20% (intermediate PR, IPR), one had 80% (high PR). All four have received octreotide LAR as a therapy component. In two cases octreotide was given upfront and conferred PFS of 18 and 8 months and, in the former- regression of hepatic lesions. The other patient also received 177 Lutetium radionuclide therapy (PRRT) upon progression, resulting in uptake normalization at an hepatic mass and stabilization of other lesions. The 3rd patient had started with induction chemotherapy and then maintained imaging-confirmed stable disease and normal function, during octreotide treatment, for three years. The fourth, HG patient, survived only 8 months. Three of the 9 patients are alive with disease on treatment, surviving 25+, 57+ and one, with 50% proliferation index and a negative DOTANOC scan, has survived 16+ months, with no targeted therapy. Conclusions: This small series illustrates the diagnostic value and therapeutic implication of SSTR scans, combined with Ki67 assessment, aiming at therapy selection for IPR subgroup, receptor positive, pulmonary LCNEC patients who can benefit from octreotide targeted therapy.

A NUP98-HOXD13 leukemic fusion gene leads to impaired class switch recombination and antibody production

Myelodysplastic syndrome is a clonal process characterized by ineffective hematopoiesis and progression to acute leukemia. Although many myelodysplastic syndrome and leukemic patients have compromised immunity, the role of underlying mutations in regulating immune function is poorly understood. Recent studies show that NUP98-HOXD13 (NHD13) fusion gene results in myelodysplastic syndrome and impairs lymphocyte differentiation in transgenic mice. In our studies, we sought to elucidate the mechanism by which NHD13 affects B-lymphocyte development and function. Based on our preliminary findings that transgenic mice had increased levels of IgM and reduced IgG1 and IgE, we hypothesized that the fusion gene might impair class switch recombination (CSR). Mice were immunologically challenged with dinitrophenol. NHD13 mice showed a marked reduction in B-lymphocyte differentiation in their bone marrow and spleen following dinitrophenol stimulation and had reduced production of dinitrophenol-specific antibodies. Spleen follicles from these mice were small and hypocellular, indicating failure of clonal expansion. When isolated NHD13 B lymphocytes were stimulated in vitro using Escherichia coli lipopolysaccharide or lipopolysaccharide + interleukin-4, they failed to undergo sufficient CSR and proliferation. Taken together, our findings show that expression of NUP98-HOXD13 impairs CSR and reduces the antibody-mediated immune response, in addition to its role in leukemia. Further delineation of the NUP98-HOXD13 transgene may reveal novel pathways involved in CSR.

Alteration of lipids and the transcription of lipid-related genes in myelodysplastic syndromes via a TP53-related pathway

Myelodysplastic syndromes (MDS) are clonal stem cell diseases of the bone marrow characterized by abnormalities in maturation of hematopoietic cells of all lineages. MDS patients frequently have lower lipids and high rates of apoptosis and p53 (TP53) expression. An association between the reduced lipids in MDS and the expression of lipid-related genes was sought. We further evaluated whether 3-hydroxy-3-methyl-glutaryl-CoA reductase (HMGcoAR) and low-density lipoprotein receptor (LDL-R) are regulated by TP53 invivo and invitro. Gene expression was measured using real-time reverse transcription polymerase chain reaction on RNA extracted from bone marrow and peripheral blood from eight newly diagnosed MDS patients and eight controls and from mice livers. Serum lipid profile was measured using colorimetric enzymatic procedures. Total- and LDL cholesterol were lower in MDS patients in comparison to controls (p = 0.04 and p = 0.01, respectively). HMGcoAR messenger RNA increased in peripheral blood and bone marrow of MDS patients compared to controls (p = 0.04 and p = 0.01, respectively). LDL-R messenger RNA was higher only in the peripheral blood of MDS patients (p = 0.05). Comparable results were obtained invivo. The transcription of these genes correlates with TP53 activation as documented by p21 messenger RNA elevation, a surrogate for TP53 activation and by using TP53 temperature-sensitive cells treated with adriamycin. To conclude, an association between reduced lipids in MDS and expression of HMGcoAR and LDL-R genes was documented. The transcription of these genes can be regulated by TP53.

Solitary mastocytoma in an adult with an unusual clinical presentation

To the Editor: A 30-year-old woman presented with a 10-year history of headaches, flushing, and anaphylaxis triggered by exercise, stress, heat, or scalp friction. Recently, she noticed a poorly circumscribed scalp mass. Computed tomography imaging confirmed a 3.3- × 1.5-cm subcutaneous mass, without bone involvement. The mass fluctuated in size (up to 10 cm), and episodes of anaphylaxis occurred with manipulation. Interestingly, the patient experienced no flushing or anaphylaxis while pregnant, but her symptoms returned after delivery. A punch biopsy specimen of the skin obtained from the edge of the tumor showed subtle histopathologic changes with a mild perivascular, perifollicular lymphocytic infiltrate and scattered mast cells (Fig 1). Surgical resection was performed and revealed a scalp mass with lobulated appearance. Histopathologically the tumor was composed of a dense monomorphous population of mast cells, with abundant granular cytoplasm; positive Giemsa and tryptase stains confirmed the diagnosis of mastocytoma (Fig 2). Postoperatively, the patient made a complete recovery and had no further symptoms at 27 months’ follow-up.Fig 2A and B, Intraoperative photographs of the solitary mastocytoma in vivo and after surgical resection. C and D, Hematoxylin-eosin sections showing dense population of mast cells with round nuclei, bland chromatin, and abundant granular cytoplasm. D (insert), There is diffuse immunoreactivity to mast cell tryptase.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Solitary mastocytoma is primarily a disease of young children, with adult cases rarely reported.1Pandhi D. Singal A. Aggarwal S. Adult onset, hypopigmented solitary mastocytoma: report of two cases.Indian J Dermatol Venereol Leprol. 2008; 74: 41-43Crossref PubMed Scopus (2) Google Scholar Lesions are typically cutaneous, involving the extremities, face, scalp, and trunk. Cutaneous mastocytomas may be associated with systemic disease, but up to 15% of patients with a mast cell disorder may develop a solitary mastocytoma without systemic involvement.2Castells M.C. Extracutaneous mastocytoma.J Allergy Clin Immunol. 2006; 117: 1513-1515Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar In our case, a diagnosis of concurrent systemic mastocytosis was suspected, but not confirmed. The bone marrow biopsy specimen showed few clusters of mast cells (>15 cells). The serum tryptase level ranged from 11 to 14 ng/mL; therefore, under the World Health Organization (WHO) threshold of 20 ng/mL. Thus, by WHO criteria, one major criterion was met (clusters of >15 mast cells), but no minor criteria were satisfied for the diagnosis of systemic mastocytosis.3Valent P. Horny H.P. Li C.Y. Longley J.B. Metcalfe D.D. Parwaresch R.M. et al.Mastocytosis.in: Jaffe E.S. Harris N.L. Stein H. Vardiman J.W. World Health Organization Classification of Tumors: Tumours of the Haematopoietic and Lymphoid Tissues. IARC Press, Lyon, France2001: 291-300Google Scholar Molecular studies showed no D816V mutation in the KIT gene and no evidence of the FEP1L1-PDGFRA fusion transcript. Adult-onset mastocytoma has a less favorable rate of spontaneous resolution compared with the childhood lesions, and surgical excision may be necessary.1Pandhi D. Singal A. Aggarwal S. Adult onset, hypopigmented solitary mastocytoma: report of two cases.Indian J Dermatol Venereol Leprol. 2008; 74: 41-43Crossref PubMed Scopus (2) Google Scholar Preoperative collaboration with the anesthesiology team is important to avoid hemodynamic instability during surgical manipulation. Our patient was premedicated with oral cetirizine. It is noteworthy that in this case the tumor became evident only years after the onset of nonspecific clinical symptoms. The initial histopathologic changes were very subtle, offering only a hint of what would later be seen in the surgical specimen. This fact may be explained by the superficial nature of the biopsy and illustrates the necessity of close follow-up and clinical-pathologic correlation when similar findings are encountered on the biopsy specimen. Interestingly, the patient had a complete lack of symptoms during pregnancy, with symptoms returning postpartum. To our knowledge, this is the first report of concurrent solitary mastocytoma and pregnancy, with complete control of mastocytoma-related symptoms during pregnancy. It has been previously reported that pregnancy may worsen the symptoms of mastocytosis.4Donahue J.G. Lupton J.B. Golichowski A.M. Cutaneous mastocytosis complicating pregnancy.Obstet Gynecol. 1995; 85: 813-815Crossref PubMed Scopus (19) Google Scholar, 5Worobec A.S. Akin C. Scott L.M. Metcalfe D.D. Mastocytosis complicating pregnancy.Obstet Gynecol. 2000; 95: 391-395Crossref PubMed Scopus (37) Google Scholar