Sergio Sanchez-Sosa

Cutaneous langerhans cell sarcoma: a case report and review of the literature

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that occurs in lymph nodes, liver, skin, spleen, lung, and bone. We report a case of LCS in a 47-year-old man with a 6-month history of scalp mass and cervical lym-phadenopathy. Clinical and pathologic data were available.A histologic examination demonstrated a proliferation of cells with malignant cytologic features. Because of its poorly differentiated morphologic features, hematologic and nonhematologic entities were ruled out by immunohistochemical screening with a broad panel of antibodies. Ultrastructural studies demonstrating Birbeck granules and consistent expression of CD1a, S-100 protein, and langerin by immunohistochemistry were helpful in identifying the Langerhans cell origin. Int J Hematol. 2007;85:116-120. doi: 10.1532/IJH97.NA0604

Treatment of vitiligo with a chimeric monoclonal antibody to CD20: a pilot study

Five patients with active disseminated vitiligo were given 1 g of a chimeric (murine/human) monoclonal antibody to CD20 in a single intravenous infusion and followed‐up for 6 months. Three of the patients showed an overt clinical and histological improvement of the disease, one presented slight improvement and the remaining patient showed no changes. Improvement was neither associated with changes in laboratory parameters nor to a specific human leucocyte antigen D‐related (HLA‐DR) phenotype. We believe that these preliminary results are encouraging, and further clinical trials should be undertaken. An important aim should be the finding of a marker with a good response to this therapeutic approach.

Tuberculosis-associated fatal hemophagocytic syndrome in a patient with lymphoma treated with fludarabine.

A patient with a stage IV high-grade non-Hodgkins lymphoma who developed a fatal hemophagocytic syndrome is presented: When the patient had achieved complete remission and receiving fludarabine and chlorambucil/prednisone, she developed miliary tuberculosis, the CD4+ T-cell count then being 50/microL; the hemophagocytic syndrome ensuing at this point was fatal. Speculations about the predisposing factors that could have led to this complication are discussed focusing on the severe cellular immunosuppression which developed probably related to the use of fludafabine: it could be useful in the future to use anti-tuberculous prophylaxis in selected patients treated with this purine nucleoside analog.

Immunophenotypic characterization of lymphoid cell infiltrates in vitiligo

The pathogenesis of vitiligo is still controversial. The purpose of this study was to gain insight into the nature of lymphoid cells infiltrating depigmented areas of skin in vitiligo. Immunochemical procedures were carried out in biopsies from 20 patients with active lesions to search for cells expressing CD1a, CD2, CD3, CD4, CD5, CD8, CD20, CD25, CD30, CD56, CD68 and CD79a. Results indicate that early lesions are infiltrated mainly by dendritic cells, whereas older lesions display significantly lower proportions of these cells and increased percentages of mature T cells. This finding might suggest that the autoimmune reactivity towards melanocyte antigens might be T cell‐dependent and antigen‐driven. It is possible that a non‐immune offence of melanocytes is responsible for the exposure of intracellular antigens, while autoreactivity might be a secondary, self‐perpetuating mechanism.

Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report.

AbstractPrimary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome.Virtual slideThe virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045.

Rhabdomyomatous mesenchymal hamartoma mimicking basal cell carcinoma.

To the Editor: We read the case report by Biswas with great attention because we had recently a similar case of rhabdomyomatous mesenchymal hamartoma (RMH), with increased number of melanocytes, mimicking clinically a basal cell carcinoma. The lesion occurred in an 80-yearold white Hispanic man presented to the dermatologic clinic with a 5-month history of a solitary 5-mm brownish-tan nodule, focally ulcerated, on the left upper eyelid. The patient had a medical history of type II diabetes mellitus, emphysema, and chronic hypertension; left endophtalmitis was diagnosed 30 years ago. The patient had no known history of congenital anomaly. He underwent excision of the lesion, based on a clinical diagnosis of basal cell carcinoma. Histologically, the lesion consisted of numerous disorganized and randomly arranged mature striated skeletal muscle fibers with cross-striations (Figs. 1A, B); pilosebaceous units were also identified. The epidermis showed focal increased number of melanocytes with hyperpigmented basal layer and ulceration. There was no evidence of nesting of melanocytes or atypia, and no malignancy was identified. The dermis revealed pigmentary incontinence and hemorrhage. Masson trichrome highlighted the skeletal muscle fibers (Fig. 1C), and immunohistochemistry examination showed that the muscle fibers were strongly positive for muscle-specific actin (Fig. 1D). We believe that the clinicopathological features of the lesion described herein are different from those of the lesions previously reported. The present case showed a recent-onset RMH, occurred in an adult man without any recognized congenital malformation. The cases reported in adult patients are scant (to the best of our knowledge 6, including the present case), and in some of them, the time of evolution is unknown. RMH etiology is not well understood, and some authors have attributed a congenital nature due to the fact that most of the cases are diagnosed during the pediatric age and some of them associated with congenital diseases; also, the resemblance of the hamartomatous tissue to the normal structures suggests an anomalous development. Hendrick et al suggested that traction of the skin may predispose toward the development of RMH. Interestingly, our case and the case in the study of Biswas are similar, both having increased number of melanocytes and absence of atypia. Biswas explained the melanocytic hyperplasia as an ‘‘inductive phenomenon,’’ a reactive melanocytic proliferation, and we agree with this hypothesis; however, we also believe that the presence of a melanocytic lesion might be a non-related or incidental finding, occurring in the setting of RMH. Particularly in our case, other melanocytic conditions including acquired melanosis should be excluded.

Urinary bladder paraganglioma in childhood: a case report and review of the literature.

Bladder paraganglioma (BP) is a rare entity and is exceedingly uncommon in childhood. Pheochromocytomas/paragangliomas are components of several hereditary cancer syndromes, and up to 30% may be associated with germ-line mutations of genes, including VHL, RET, and SDH. We present a 16-year-old female who was admitted with macroscopic hematuria and anemia. A cystoscopy demonstrated a polypoid and hemorrhagic mass arising from the floor of the bladder. She underwent a transurethral resection of clinically suspected urothelial papilloma. A histologic examination of the tumor showed large polygonal cells with eosinophilic cytoplasm, arranged in a zellballen pattern, surrounded by a fibrous network. Immunohistochemical studies showed a strong expression of neuroendocrine markers and lack of reactivity for epithelial markers. The diagnosis of BP was established; eight months later, a recurrence was observed and the patient underwent a partial cystectomy. Our case represents the 1st BP in childhood reported in the literature with absent SDHB staining by immunohistochemistry. We discuss the clinical and pathologic findings and present a review of BP in childhood.

Ruxolitinib Chronic Myelomonocytic Leukemia-Associated Myelofibrosis:A Case Report

Myelodysplastic syndromes (MDS) are a heterogenous group of clonal stem cell disorders which generally occur in older adults but may also affect children. Primary MDS should be distinguished from secondary MDS associated with antineoplastic or immunosuppressive therapy (t-MDS), exposure to toxic compounds, or genetic disorders [1]. Chronic myelomonocytic leukemia (CMML) has been considered a variant of MDS [1] and fibrotic forms have been observed in up to 16% of cases [2]. Accordingly, the histologic evaluation of a trephine bone marrow biopsy is of critical importance for the evaluation of fibrotic or hypocellular MDS since these patterns are not reflected by the cytological examination.

Basal cell carcinoma of the prostate gland: a case report and brief review of the basal cell proliferations of the prostate gland.

Basal cell proliferations within the prostate gland encompass a group of benign and malignant entities. Although basal cell hyperplasia is a common finding, basal cell carcinoma of the prostate gland is a rare tumor that can be mistaken by a benign condition and represents a diagnostic problem in genitourinary pathology. We report a case of basal cell carcinoma in a previously healthy 65-year-old man with urinary symptoms and low prostate-specific antigen. The microscopic findings are presented and the use of immunohistochemical markers classifying basal cell lesions of the prostate discussed.

Skin Biopsy Diagnosis of Langerhans Cell Neoplasms

This chapter reviews the clinical presentation, histopathology, immunoprofile and molecular features of Langerhans cell neoplasms of the skin including Langerhans cell histiocytosis (LCH) and its malignant counterpart, Langerhans cell sarcoma (LCS). Biopsy of the skin is a useful method to confirm LCH/LCS diagnosis, as cutaneous involvement is seen in more than 50% cases. Skin can be the only presenting site of LCH, but it is usually seen as an integral part of multisystemic disease involvement.