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Background Therapy-related acute monocytic leukemias in patients with plasma cell dyscrasias are infrequent. Case presentation We here present a case of a 60 year old female who developed an acute monocytic leukemia two years after the diagnosis of multiple myeloma. She was treated with an alkylating agent and bortezomib before undergoing a hematopoietic stem cell transplantation. She suffered of multiple severe infections until her immune system was adequately reconstituted. A year afterwards, she presented signs of deterioration unrelated to the MM, with pancytopenia. The bone marrow aspirate failed to show a prominent blast population. The diagnosis of AML was confirmed after a bone marrow biopsy. Discussion The development of acute leukaemia after treatment for multiple myeloma is a well characterized phenomenon. Most frequently, patients develop a myelomonocytic leukemia. Similarly, synchronous acute myeloid leukemias are myelomonocytic or myeloblastic. Rarely synchronous AMLs are monocytic. The development of such suggests a dysfunctional bone marrow microenvironment.

Epidemiology of polycythemia vera (PV) is derived from cohort studies in Western populations. PV studies in the Latin population are limited and have described a lower prevalence of JAK2 mutation. A retrospective cohort of 90 Mexican patients from a single center with PV according to the 2016 World Health Organization diagnostic criteria was studied to establish clinical presentation in Mexicans and evaluate risk factors for mortality and vascular events. At diagnosis, the median age was 59 years; half were females, 90.7% harbored the JAK2 V617F mutation, and 7.1% had an exon 12 mutation. Arterial and venous thromboses were observed in 13.3 and 27.8% of patients, respectively. Seventy-one patients received hydroxyurea and one interferon alpha-2a, of which 51.5% achieved a complete response and 25.0% a partial response. The incidence rate for postdiagnosis arterial thrombosis was 2.68 cases per 100 person–years, for venous thrombosis 2.84, for major hemorrhage 2.57, for progression to myelofibrosis 1.14, and for leukemic transformation 0.44. The mortality rate was 3.54 cases per 100 person–years, and median of survival was 150 months. In multivariable analysis, survival was adversely affected by leukocytosis ≥12 × 109/L (p = 0.013); thrombocytosis ≥450 × 109/L was a risk factor for arterial thrombosis (p = 0.023), and anticoagulation at diagnosis for major hemorrhage (p = 0.049). In conclusion, epidemiology of PV in Mexican population is similar to that reported in Western studies, but with more venous thrombosis at diagnosis and a higher incidence of vascular complications postdiagnosis.

Background Thrombotic events are well documented in primary erythrocytosis, but it is uncertain if secondary etiologies increase the risk of thrombosis. This study aimed to determine the causes of erythrocytosis and to identify its impact as a risk factor for thrombosis. Methods Data were obtained from patients with erythrocytosis between 2000 and 2017 at a referral hospital in Mexico City. Erythrocytosis was defined according to the 2016 WHO classification. Time to thrombosis, major bleeding, or death were compared among groups of patients defined by the etiology of erythrocytosis using a Cox regression model, adjusting for cardiovascular risk factors. Results In total, 330 patients with erythrocytosis were studied. The main etiologies of erythrocytosis were obstructive sleep apnea (OSA) in 29%, polycythemia vera (PV) in 18%, and chronic lung disease (CLD) in 9.4% of the patients. The incidence rate of thrombosis was significantly higher in patients with PV and CLD than that in patients with OSA (incidence rates of 4.51 and 6.24 vs. 1.46 cases per 100 person-years, P=0.009), as well as the mortality rate (mortality rates of 2.72 and 2.43 vs. 0.17 cases per 100 person-years, P=0.003). Conclusion The risk of thrombosis in CLD with erythrocytosis was comparable to that in patients with PV. Further larger-scale studies are needed to confirm these findings and evaluate the benefits of preventive management of COPD with erythrocytosis similar to PV.

Acroangiodermatitis (AAD) is often seen in association with various vascular anomalies such as venous insufficiency, vascular syndromes, and conditions associated with thrombosis. This is the first case reported in the literature associated with arteriovenous fistula stenosis in a patient with chronic kidney disease on hemodialysis. This case is being described for its rarity and to familiarize the clinicians with this unusual complication, especially, to prevent them from thinking of this condition as an infectious complication. It is essential to recognize the uniqueness of the pathophysiology of this disease and to do a clear distinction with that of a venous ulcer. With this work we also aim to help health practitioners with proper management of the condition. As we've seen, surgical treatment in appropriately selected cases corrects the reflux of the venous system and successfully improves the appearance of the verrucous lesion. Our patient was successfully treated by correcting the arteriovenous fistula stenosis with near-complete subsidence of the verrucous lesion within days of the procedure. Acroangiodermatitis management must be conducted with a multidisciplinary approach (dermatology, vascular surgery, and internal medicine). It is essential the comprehensive management of these patients, to ensure prompt recovery and avoid chronic effects, as well as to guarantee the quality of life in the future.

The histopathological diagnosis of dermal-based lymphoid infiltrates and proliferations is often challenging due to the vast list of biologically diverse entities that archetypally or occasionally center in the mid-dermis, especially because significant overlap exists in their clinical, histopathologic, and immunophenotypic features. The differential diagnosis includes reactive infiltrates in common and rare inflammatory dermatoses, benign conditions that may mimic lymphoid neoplasms (pseudolymphomas), and true clonal proliferations arising either primarily in the skin or rarely in extracutaneous tissues with secondary cutaneous dissemination. While numerous histopathological and immunophenotypic features have been reported to support a definitive diagnosis, no single ancillary test is sufficient for their distinction. Therefore, in this review we advocate a stepped histopathological approach for dermalbased lymphoid infiltrations, employing as key elements the general lymphocytic composition (relative B- versus T-cell ratio), coupled with the predominant cytomorphology (cell size) present. Following this strategy, the relative incidence of cutaneous involvement by each disease should always be considered, as well as the notion that a definitive diagnosis must be founded on a multiparameter approach integrating all clinical, histopathologic, immunophenotypic, and-in selected cases-molecular features.

We report the case of a 26-year-old woman with a 19 cm malignant hepatic neoplasm with morphological features that closely resembled a follicular thyroid carcinoma. Despite this, it was interpreted as a cholangiocarcinoma due to the absence of a primary thyroid tumor and the lack of thyroglobulin and TTF-1 immunoreactivity by the hepatic tumor. The left hepatic lobectomy specimen showed an encapsulated and multinodular gray-white mass with cystic and hemorrhagic areas. Microscopically, it displayed predominant macro and microfolicullar patterns with focal solid, trabecular and insular areas. The small and distended follicles contained a colloid-like secretion and were lined by low cuboidal cells with scant cytoplasm, round or oval hyperchromatic nuclei with fine chromatin. The solid areas, trabecular and insular structures were similar to those of follicular or papillary thyroid carcinomas. In addition, some of the neoplastic cells had clear nuclei with occasional grooves. The tumor was positive for cytokeratin (CK) 7, CK 19 and CD138, and negative for TTF-1, thyroglobulin, Hepar-1, Glypican-3, alpha-fetoprotein and neuroendocrine markers. A thyroid neoplasm was excluded clinically and by ultrasound and computed tomography. Although, the residual hepatic parenchyma was initially not cirrhotic, the patient eventually developed cryptogenic cirrhosis. The patient received adjuvant chemotherapy and died of metastatic disease 18 months after surgery. The thyroid-like pattern broadens the morphologic spectrum of cholangiocarcinoma.

We described 7 examples of Merkel cell carcinoma of the skin with eccrine and squamous differentiation. Five patients were men, and 2 were women; and their ages ranged from 63 to 81 years (mean age, 73 years). Six tumors arose in the head and neck; and one, on the sole of the right foot. Three tumors recurred locally, and 2 metastasized to the regional lymph nodes. No patient developed distant metastasis. Two patients died of unrelated causes. Five Merkel cell carcinomas showed classic cytology, and 2 were similar to small cell carcinomas of the lung. All 7 tumors showed small eccrine ducts, and 2 exhibited foci of squamous differentiation. The eccrine ducts label with cytokeratin 7 and carcinoembryonic antigen, whereas the predominant endocrine component displayed the characteristic paranuclear dot-like reactivity with cytokeratin 20 and was synaptophysin and chromogranin positive. The lymph node metastasis contained both eccrine ducts and squamous elements, suggesting that they are an integral component of the tumors. Eccrine differentiation in Merkel cell carcinomas similar to small cell carcinomas of the lung and extrapulmonary sites is an important feature in the differential diagnosis because eccrine differentiation has not been described in primary or metastatic small cell carcinomas. The prognosis of these Merkel cell carcinomas with divergent differentiation appears to be less aggressive than that of pure Merkel cell carcinomas. However, larger series of patients with longer follow-ups are needed to confirm this observation.

Foamy gland adenocarcinoma is a variant of pancreatic ductal carcinoma, whose precursor has not been described. We describe here the morphologic and immunohistochemical features of the pancreatic intraepithelial neoplasia (PanIN) lesions associated with invasive foamy pancreatic adenocarcinoma. The staining properties and morphologic and immunohistochemical features of 3 foamy PanIN lesions were compared with those of 7 pancreatic foamy gland adenocarcinomas. Hematoxylin and eosin, Mayer mucicarmine, periodic acid-Schiff, and Alcian blue stains were available for review in all cases. Immunohistochemical labeling for cytokeratin (CK)7, CK20, carcinoembryonic antigen polyclonal, MUC1, MUC2, CDX2, p53, and cyclin D1 was performed. The PanIN-1 lesions were found in the nonneoplastic pancreas and were similar to the PanIN-1 lesions of ordinary pancreatic ductal carcinoma. The PanIN-2 and -3 lesions were recognized immediately adjacent to or within the invasive foamy gland carcinoma. In these lesions, small or markedly dilated ducts were lined by cuboidal and columnar dysplastic nonfoamy cells and foamy cells. Hobnail cells were present in 2 cases. The PanIN-1, 2, and 3 lesions and the invasive foamy gland adenocarcinomas stained with mucicarmine, periodic acid-Schiff, and Alcian blue. The 3 PanIN-2 and -3 lesions and all 7 invasive foamy adenocarcinomas labeled with CK7, carcinoembryonic antigen polyclonal, and MUC1, whereas only 2 PanIN-2 and -3 lesions and 5 invasive adenocarcinomas showed immunoreactivity for cyclin D1 and p53. Three distinctive foamy PanIN lesions were identified within 7 invasive foamy gland pancreatic adenocarcinomas. The gradual progression of cytological and architectural abnormalities of the PanIN lesions from PanIN-1 to PanIN-3 excludes neoplastic ductal spread. These foamy PanIN lesions probably represent cancer precursors.

Main findings We report what to our knowledge is the first case of a giant multicystic cystadenoma of the Cowper's glands. An otherwise healthy 41-year-old man presented with acute urinary retention. Physical examination showed a perineal mass. Different imaging techniques demonstrated a multicystic tumor and en bloc excision was performed. Histological evaluation showed that the tumor arised from the bulbourethral glands; immunohistochemistry proved positive staining for high molecular weight cytokeratin. Case hypothesis Cystic tumors in the pelvis can arise from different structures. Malignancy should be ruled out. Surgical excision can be diagnostic and curative. Future implications When evaluating a pelvic cystic tumor, Cowper's glands cystadenoma may be a differential diagnosis and must be considered. Similar to prostate cystadenomas, en bloc excision is the optimal treatment.

Background: Rosai–Dorfman disease (RDD) is an uncommon, benign histiocytic proliferative disorder of unknown origin. It predominantly affects the lymph nodes, but can also be found extranodal in different organs. Nervous system involvement is rare, and the most cases are intracranial. Surgical treatment is indicated when the central nervous system (CNS) in compromised. Case Description: We herein describe the management of a 27-year-old woman who presented progressive spinal cord symptoms, secondary to an isolated intramedullary lesion, which had a histological confirmation of RDD. To our knowledge, this is the 6th case reported in English written manuscripts. We review these cases and analyze some of the literature concerning the disease. Conclusions: RDD shows some variability in the involvement of the entire neuraxis, and because its ability to mimic meningeal and primary brain tumors, it is essential to be aware of this entity and consider RDD in the differential diagnosis of various lesions of the CNS. The conclusive diagnosis must be obtained by histological methods, so surgical approaches have to be discussed. Although it is not considered as a malignancy, options for postoperative medical treatment are variable and include radiation, chemotherapy or maybe monoclonal antibodies for refractory or recurrent cases.