Solitary Skin Lesion Research Articles

Solitary mastocytoma presenting at birth.

Mastocytosis is a rare disease which occurs in both children and adults, and it can manifest as a solitary or multiple skin lesions. Both can cause cutaneous or systemic symptoms. Because of the heterogeneity of clinical presentation of mastocytosis and its rare prevalence, it can be hard to suspect the mastocytosis at the first time. Most solitary mastocytomas are about 1–5 cm in diameter and have features of brownish-yellow, minimally elevated plaques with a smooth shiny surface. This article presents a case of solitary mastocytoma which occurred in neonate and that we treated through surgical excision. In histopathological examination, it consisted of c-kit-positive mast cells. Although pediatric cutaneous mastocytosis might regress spontaneously, clinicians should keep in mind that it could be associated with systemic mastocytosis which involves hematopoietic system.

Childhood leprosy in the postelimination era: A vision achieved or a concern growing at large

Background: The proportion of childhood leprosy has been gaining importance of late as a performance indicator of the National Leprosy Eradication Program. A sizable proportion of the newly detected cases comprises children. The prevalence rate has been used as a measure of existence of infection though leprosy has attained elimination levels in the country. Materials and Methods: A retrospective descriptive study was conducted. Case records of children newly diagnosed with leprosy aged <15 years between April 2005 and October 2015 were studied and included in the study. Findings such as patches, nerve thickness, spectrum of leprosy, grade of deformity, smear status, presence of reaction, histopathological diagnosis, and treatment history were noted. Results: The percentage of childhood leprosy among the newly diagnosed cases in our study was found to be 11.2%. A solitary skin lesion was the most frequent manifestation in 64.4% children. The second most common finding seen in these patients included the presence of thickened nerves in 46.6% children. Smear showed acid-fast bacilli in 8.8% cases. Conclusion: There is an urgent need for improving clinical acumen in diagnosing childhood leprosy. The importance of appropriate and complete treatment along with meticulous surveillance in endemic areas and looking for household contacts needs to be stressed on.

Primary Cutaneous CD4+ Small/Medium T-cell Lymphoproliferative Disease: A Case Report

Primary cutaneous lymphoma is a heterogeneous group of diseases. Primary cutaneous CD4+ small/medium Tcell lymphoma (CD4+ SMTCL) is one of the rare subtypes; representing only 2-3% of all cutaneous lymphomas. It was recently described as lymphoproliferative disorder because of its indolent clinical behavior and many similarities with cutaneous pseudolymphoma. This disease is a provisional entity according to latest World Health Organization (WHO) classification of hematolymphoid tumors. CD4+ SMTCL has a n excellent 5-year survival of 60-85%. CD4+ SMTCL usually present with a solitary skin lesion of the head and neck region, and most of reported cases were treated with local managements only. The common clinical presentations and histological features are still not well understood, and no optimal therapy is established. We report a case of primary cutaneous CD4+ small/medium Tcell lymphoma, our patient presented with a facial skin lesion that was treated with excision only.

Recommendations for the Optimal Radiation Dose in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma: A Report of the Dutch Cutaneous Lymphoma Group

To determine the optimal radiation dose for treatment of primary cutaneous anaplastic large cell lymphoma (C-ALCL) with solitary or localized, multifocal or recurrent skin lesions. In this multicenter study, patients with C-ALCL who had been treated with radiation therapy (RT) between 1984 and 2016 were retrieved from the Dutch registry of cutaneous lymphomas. Distinction was made between patients first presenting with solitary or localized lesions (n=63), with multifocal skin lesions (n=6), and patients with a skin relapse (n=22). Radiation doses, treatment response, and follow-up were evaluated. Radiation doses were categorized as low-dose (≤20Gy), intermediate-dose (21-39Gy), and high-dose (≥40Gy) RT. Of 63 patients presenting with solitary or localized skin lesions, 61 (97%) showed a complete response (CR). There were no differences in CR between low-dose (16 of 17), intermediate-dose (15 of 15), and high-dose RT (30 of 31). After a median follow-up of 46months, 30 of 63 patients (48%) had a relapse, but in-field relapses were never observed. Six of 6 patients (100%) initially presenting with multifocal skin lesions showed a CR (3 of 3 low-dose, 2 of 2 intermediate-dose, 1 of 1 high-dose RT). After a median follow-up of 27months, 3 of 6 patients had a relapse. Treatment of 33 skin relapses in 22 patients showed no differences in CR between low-dose (18 of 19), intermediate-dose (6 of 6), and high-dose RT (8 of 8). In the last 10years there has been a decrease in radiation dose used in the treatment of C-ALCL. Treatment of multifocal and recurrent lesions with a dose of 8Gy (2×4Gy) resulted in CR of 17 of 18 lesions. Our results show that a radiation dose of 20Gy (8×2.5Gy) is effective in patients presenting with solitary or localized skin lesions. For patients with multifocal skin lesions and patients with a skin relapse, a dose of 8Gy (2×4Gy) may be sufficient.

Congenital Embryonal Rhabdomyosarcoma Presenting as a Cutaneous Nodule in a Neonate

Rhabdomyosarcoma is the commonest malignant soft tissue sarcoma affecting children. It originates from the embryonic mesenchyme precursor of striated muscle and involves the head and neck region as a solitary or multiple skin lesion. A 20-day-old female neonate presented with a cutaneous nodule over left shoulder. Hematoxylin and eosin staining on the biopsy specimen showed malignant small round cell tumor. Immunohistochemistry studies were positive for myogenin (nuclear positivity) and desmin (cytoplasmic positivity). Presentation in the neonatal period is rare and poses a diagnostic and therapeutic challenge. The management involves complete excision of the tumor followed by chemotherapy. Int J Clin Pediatr. 2016;5(3-4):47-50 doi: https://doi.org/10.14740/ijcp254e

Patient Characteristics, Locations and Histopathological Features of Pilomatrixomas in Erzurum/Turkey

Aim: The aim of this study is to examine the patient characteristics, locations, presentations and histopathological features of pilomatrixomas in a group of patients. Materials and methods: We analyzed 41 patients that histopathologically diagnosed as pilomatrixoma in Erzurum Regional Training and Research Hospital between 2009 ad 2014, retrospectively. Results: Patient’s mean age was 30 years (age range: 3–78 years), with a female to male ratio of 26:15. There were 12 patients in the pediatric age group (<16 years). The average diameter of the lesion at presentation was 18.5 mm (range: 2–55 mm). A total of 36 out of 41 (87.8%) patients presented with solitary lesion, while 5 patient (12.2%) had two lesions. Most common location in our patient group was head and neck region and second most common location was limbs. The most common clinical presentation was a painless solitary skin lesion which was noticed incidentally. All cases more or less shared the same microscopic features of pilomatrixoma such as basophilic cells, eosinophilic shadow cells, calcification and foreign body type giant cells. Conclusion: Pilomatrixoma is not an uncommon benign lesion. It is more common in female gender, adult age group and at the head and neck region.

Quantified ultrasound elastography in the assessment of cutaneous carcinoma.

To evaluate the feasibility of high-frequency ultrasound and ultrasound elastography (USE) in discriminating benign from malignant skin lesions in a prospective cohort study and to introduce the use of a "strain ratio" for evaluation of skin lesions. A commercial ultrasound system with a 14-MHz transducer was used to visualize skin lesions requiring biopsy on clinical evaluation. Anatomic ultrasound and USE imaging of the skin lesions was performed using 2- to 4-mm gel stand-off pads. A region of interest was manually selected over the area of each lesion with the lowest strain. The concept of a strain ratio of the compressibility of the normal skin at the corresponding layer to that of the least compressible region of a lesion in question was created and applied. This ratio was subsequently correlated with blind histopathological evaluation for malignancy. 55 patients were included in the study with a total of 67 lesions evaluated. 29 lesions were malignant and 38 benign. All malignant lesions had strain ratios ≥3.9. All benign lesions had strain ratios ≤3.0. A diagnostic value between 3.0 and 3.9 would result in 100% sensitivity and specificity in the characterization of these lesions as malignant. This pilot study demonstrated that USE plus strain ratio appears to be a promising modality in providing diagnostic determination between cancerous and benign primary solitary skin lesions prior to biopsy. This is the first reported study applying an original mathematical elastographic ratio, or strain ratio, to evaluate primary solitary skin lesions.

Rare Adverse Effect of Treatment With Minocycline

An 18-year-old woman presented with a 4-week history of a solitary painful bullous skin lesion over the lower right anterior tibia accompanied by generalized myalgia, polyarthalgia, joint stiffness, paraesthesia, and episodic ankle swelling. The lesion first appeared as a skin-coloredpapule and,within aweek, developedanerythematoushalo.Hermedical history includes asthma and acne. To our knowledge, there is no family history of this condition. Her medications included minocycline hydrochloride (for 25 months for acne), ranitidine hydrochloride, contraceptive pills, and iron supplements. An initial physical examination revealed mild synovitis in both ankles and an erythematous, raised papule overlying the distal aspect of the right anterior tibia. Treatment with prednisone acetate (10 mg daily) was initiated. Seven weeks later, the original skin lesion evolved into an ulcer with erythematous, raised borders (Figure 1). The case patient had new eruptions of nonindurated, violaceous, nontender, reticulated patches overlying the skin on the bilateral anterior tibias, consistent with livedo reticularis. She denies any previous episodes, and the results of a review of mitigating factors were negative. A laboratoryworkup revealed apositive antinuclear antibody 1:640nucleolar pattern, anelevatederythrocyte sedimentation rate, andelevatedC-reactiveprotein andserum IgG levels. The results of the following laboratory studies were negative or normal: complete blood cell count, urinalysis, liver profile, Lymedisease, parvovirus B19,Monospot test, uric acid, rheumatoid factor, antistrepolysin antibody, anti-Scl70, antihepatitis C virus, hepatitis B surface antigen, hepatitis B core antibody, cytomegalovirus antibody, and human T-lymphotropic viruses 1 and 2 antibody. Culture results of the skin lesion were negative. The results from chest radiography were normal. Figure 1.Ulcer with raised border and erythematous halo in distal right shin.

Extrafacial indolent CD8‐positive cutaneous lymphoid proliferation with unusual symmetrical presentation involving both feet

Indolent CD8+ cutaneous lymphoid proliferation represents a recently described entity among cutaneous T-cell lymphomas that typically presents with solitary skin lesions on the face or at acral sites and usually follows an indolent clinical course. Histopathologically, this entity is characterized by a dense dermal infiltrate of non-epidermotropic, small- to medium-sized pleomorphic CD8+ T-cells of the non-activated cytotoxic phenotype showing a clear-cut grenz zone and a low proliferation index. Distinction from otherwise aggressive T-cell lymphomas bearing a cytotoxic CD8+ phenotype is fundamental. We herein present an unusual case of indolent CD8+ cutaneous lymphoid proliferation presenting in bilateral symmetrical distribution on both feet and lacking the otherwise described grenz zone. Our case widens the spectrum of possible clinical and histomorphological variations of this entity. Taking into account the distinctive and unique clinical and microscopic features of all hitherto published cases of indolent CD8+ cutaneous lymphoid proliferation we suppose that this lymphoma subtype has to be included as a new and distinct entity in the World Health Organisation (WHO)-/European Organisation for Research and Treatment of Cancer (EORTC)-classification of cutaneous lymphomas.

Juvenile xanthogranuloma of skull base. Case report and review of the literature

Dear Editor, Juvenile xanthogranuloma (JXG) is a histiocytic nonproliferative disease of the non-Langerhans cell group. It is usually characterized by solitary or multiple skin lesions. Extracutaneous manifestations of JXG without systemic involvement are uncommon, and isolated JXG involving the skull base is extremely rare [7, 8]. We report a 3-month old patient who presented with a large mass eroding the left temporoccipital bone. A three-month old boy was referred to our hospital for a left retroauricular tumor. MRI showed an intracranial extradural retromastoid lytic lesion that was isointense on T1 and T2, and showed a slight enhancement with contrast injection (Fig. 1a). The lesion destroyed the temporal bone and displaced the left cerebellar hemisphere, producing supratentorial ventricular dilatation. Neurological examination showed no other abnormalities. Given the solitary character of the tumor and its size and location, and destructive characteristics it was decided to perform resective surgery. A retrosigmoid approach was carried out and a subperiosteal tumour was found. The lesion was completely extradural. A complete macroscopic resection was made. Histologic examination of the surgical specimen showed evidence of bone and soft tissue. The tumour was composed of histiocytic mononuclear cells accompanied by multinucleated giant cells of osteoclast and Touton types. Multinuclear and mononuclear cells were positive for CD68 and negative for CD1a and S-100 protein. Histological and immunohistochemical findings confirmed the diagnosis of JXG. A control MRI performed within 24-h post-surgery showed no residual tumour or any contrast uptake (Fig. 1b). Based on the benign nature of the tumor and after complete macroscopic resection no further treatment was given and the patient was discharged. At most recent follow-up at 3 years after surgery, the patient remains asymptomatic and free of pathology to MRI. The first report documenting single, and occasionally multiple, cutaneous lesions occurring in infants and showing spontaneous involution was written by Adamson in 1905 [1]. In 1954 Helwig and Hackney proposed the name “juvenile xanthogranuloma” to define cutaneous lesions that arise from fibrohistiocytes [8]. Juvenile xanthogranulomas have been described as solitary or multiple swellings that are nodular or plaquiform and yellow to yellowish-brown in colour. Their size varies frommillimeters to a few centimeters in diameter. As the name implies, these lesions generally occurs in the first 4 years of life [7]. The most frequently involved organ is the skin, and these tumors aremore likely to present in the head and neck. However, there are reports that document juvenile xanthogranuloma cases involving extracutaneous sites like the eyes, heart, pericardium, liver, lungs, spleen, testes and oral cavity [7]. Also documented in the literature are skeletal JXG manifestations in the context of systemic cases, but solitary osseous lesions are rare [4, 5, 13]. Involvement of the brain and its coverings are also reported [9, 12]. More often they arise in the sellar region [2, 10, 11]. Xanthogranuloma of the sella should be included in the differential diagnosis of a pediatric tumor of the sellar region. Often, xanthogranuloma is difficult to differentiate from craniopharyngioma and Rathke s cleft cyst based only on the findings of MR imaging [11]. Histopathological examination of JXG shows wellcircumscribed nodules with dense infiltrates of histiocytes. Variable numbers of multinucleated giant cells and, in 85 % of cases, Touton giant cells are present [3]. Although the appearance of histiocytic disorders may be quite similar, their P. Puerta (*) : S. Candela :G. Garcia Fructuoso Department of Neurosurgery, Sant Joan de Deu Hospital, Paseo Sant Joan de Deu, 2, 08950, Esplugues de Llobregat, Barcelona, Spain e-mail: puertaroldan82@gmail.com

Postelimination status of childhood leprosy: report from a tertiary-care hospital in South India.

Introduction. Leprosy, a statistically “eliminated” disease from the globe, continues to linger around in its endemic countries including India. Objective. This study describes the epidemiological and clinicopathological pattern of the disease seen in children over a period of 8 years following its elimination in India. Materials and Methods. Medical records of all leprosy cases up to 14 years of age registered between April 2005 and March 2013 were retrospectively analyzed. Data were retrieved using a predesigned proforma and entered into the database system for analysis. Results. Child proportion of newly registered leprosy cases did not show a significant decline in the years following its elimination. The disease seemed to manifest frequently in older children with an insignificant gender predilection. More than half of child cases had a history of household contact. Paucibacillary leprosy dominated in them with a solitary skin lesion as the most frequent presentation. Although nerve thickening was seen in nearly half of these children, neuritis and lepra reactions were less common. Deformity at the time of diagnosis was noted in 13.89% of cases. Although smear positivity was not a common feature in children affected with leprosy, a good clinicohistopathological correlation was observed in those who underwent biopsy. Conclusion. Our study and reports from different parts of the country depict the unturned curves in the epidemiology of childhood leprosy which mirrors active transmission in the community, lacunae in diagnosis, and the need to strengthen contact screening activities in the pediatric population to sustain elimination.

Fibroblastic Connective Tissue Nevus

Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign cutaneous mesenchymal lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further characterize the clinicopathologic spectrum and immunohistochemical features of this entity. Sixteen patients were female (64%) and 9 were male (36%), with age at presentation ranging from 1.5 months to 58 years (median, 10 y). Most patients presented with a solitary, slowly growing, painless plaque-like or nodular skin lesion. Eleven cases (44%) arose on the trunk, 9 (36%) on the head and neck, and 5 (20%) on the limbs. The lesion was present for a median duration of 11.5 months (mean, 13.2 mo). Grossly, the lesions were tan-brown to tan-white, smooth, and firm. Their size ranged from 0.3 to 2.0 cm in greatest dimension (mean size, 0.67 cm; median, 0.6 cm). All tumors showed poor circumscription and were situated primarily in the reticular deep dermis, extending into the superficial subcutis in 13 cases (52%). The lesion was associated with papillomatous epidermis in 17 cases (70%) and the presence of adipose tissue in the reticular dermis in 14 cases (60.9%). All tumors were composed of a proliferation of bland intradermal fibroblastic/myofibroblastic cells with indistinct palely eosinophilic cytoplasm and tapering nuclei, with no significant cytologic atypia or pleomorphism, arranged in short-intersecting fascicles and entrapping appendages. No mitoses were identified. Immunostains showed positivity for CD34 in 20 of 23 cases (87%) and weak focal positivity for smooth muscle actin in 9 of 19 cases (47%). No case stained positively for desmin or S100 protein. Clinical follow-up was obtained for 14 patients (median duration, 4 y). No tumor recurred locally, even when surgical excision was incomplete. No lesion metastasized. FCTN occurs most commonly as a plaque on the trunk and head/neck of children, involves deep dermis and superficial subcutis, and stains mainly for CD34. FCTN most likely represents a localized developmental dermal anomaly; it is entirely benign and should not be confused with dermatofibrosarcoma protuberans or other neoplasms such as dermatomyofibroma.

Subcutaneous panniculitis-like T cell lymphoma with mesenteric involvement

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare type of lymphoma, which is characterized by distinctive rimming of neoplastic lymphocytes surrounding individual adipocytes in subcutaneous tissue. SPTCL is confined to subcutis and dissemination of SPTCL to extracutaneous sites is extremely rare, if present at all. Here we present a case of SPTCL with mesenteric involvement. The patient initially presented with a solitary skin lesion at the lower extremity. A few months later, he developed new skin lesions at the abdominal wall and mesenteric lesions in the abdominal cavity. Biopsy showed SPTCL in subcutaneous tissue with mesenteric involvement. To our knowledge, this is the first case report of pathologically proven dissemination of SPTCL to other sites.

Isolated cutaneous manifestation of IgG4-related disease

AimsThe cutaneous manifestation of IgG4-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG4-positive plasma cell infiltration in...

Clinical spectrum of primary cutaneous CD30‐positive anaplastic large cell lymphoma: an analysis of the Mannheim Cutaneous Lymphoma Registry

Primary cutaneous CD30(+) anaplastic large cell lymphomas (C-ALCL) have indolent clinical behavior with an estimated 5-year survival rate of 95%. The clinical features and disease courses of C-ALCL identified in the lymphoma registry of Mannheim University hospital are described in the following. All C-ALCL patients identified in the database were analyzed in regard to clinical picture, histology, immunohistochemistry, molecular biology, staging, therapy, follow-up, and outcome. 14 C-ALCL patients were identified. The mean age was 69 years and 57% were men. Solitary skin lesions in one anatomical region were seen in 12 patients upon initial diagnosis. Two patients presented with multiple lesions at different anatomical sites. In 2 patients there was specific lymph node involvement. In one C-ALCL patient, follow-up over 17 months revealed extracutaneous infiltration. Half of the patients relapsed and 36% had multiple episodes. The majority of our patients were treated with surgical excision followed by electron beam radiotherapy. The 5-year survival rate was 93% in C-ALCL. The clinical presentation of C-ALCL varies. Staging procedures and a close clinical pathological correlation at initial diagnosis are essential. Due to a high rate of relapses and the possibility of developing extranodal manifestations over the course of the disease, close follow-up is recommended.

FGFR3, PIK3CA and RAS mutations in benign lichenoid keratosis

Benign lichenoid keratoses (BLKs) are solitary skin lesions which have been proposed to represent a regressive form of pre-existent epidermal tumours such as solar lentigo or seborrhoeic keratosis. However, the genetic basis of BLK is unknown. FGFR3, PIK3CA and RAS mutations have been shown to be involved in the pathogenesis of seborrhoeic keratosis and solar lentigo. We thus investigated whether these mutations are also present in BLK. After manual microdissection and DNA isolation, 52 BLKs were screened for FGFR3, PIK3CA and RAS hotspot mutations using SNaPshot(®) multiplex assays. We identified 6/52 (12%) FGFR3 mutations, 10/52 (19%) PIK3CA mutations, 6/52 (12%) HRAS mutations and 2/52 (4%) KRAS mutations. FGFR3 and RAS mutations were mutually exclusive. One BLK showed a simultaneous PIK3CA and HRAS mutation. In nine BLKs with a mutation, nonlesional control tissue from the epidermal margin and the dermal lymphocytic infiltrate were wild-type, indicating that these mutations are somatic. To demonstrate that these findings are specific, 10 samples of lichen planus were analysed without evidence for FGFR3, PIK3CA or RAS mutations. Our results indicate that FGFR3, PIK3CA and RAS mutations are present in approximately 50% of BLKs. These findings support the concept on the molecular genetic level that at least a proportion of BLKs represents regressive variants resulting from former benign epidermal tumours such as seborrhoeic keratosis and solar lentigo.

Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Study of 12 Cases

Abstract 5217 Objective:Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare and distinct type of T-cell lymphoma. The objective of this study was to explore the clinical presentation, treatment, and prognosis of patients with SPTCL. Methods:Twelve cases of SCPTCL, treated in our hospital between June 2005 and June 2010, were included in this study. Their clinicopathological data were reviewed and analyzed retrospectively. Results:The median age at diagnosis was 41 years (range 25–69 years) and 7 (58%) were women. 9 cases had a CD3+, CD4-, CD8- phenotype; 2 cases, a CD3+, CD4-, CD8+ phenotype; and 1 case, a CD3+, CD4+, CD8- T-cell phenotype. In all cases, strong expression of cytotoxic proteins (granzyme B, TIA-1, perforin) was observed. CD56 was expressed in 8 of 12 cases.4 patients had presented with solitary or localized skin lesions. Ulceration was observed in 3 patients. B symptoms, such as fever, chills, night sweats, and weight loss, had been recorded in 7 of 12 patients. Laboratory abnormalities, mainly anemia, leucopenia, thrombocytopenia or combined cytopenias, and elevated liver function tests and lactate dehydrogenase, were reported in 4 patients. Bone marrow examination showed histiocytic hyperplasia, hemophagocytosis, or decreased cellularity in 3 cases, but no evidence of lymphoma. A HPS was diagnosed in 4 of 12 patients (33%), and was fatal in 2 of them.Four patients presenting with solitary or localized skin lesions had been treated with radiotherapy (2 cases) or surgery (2 cases). All 4 patients reached complete remission and only 1 of them showed a skin relapse, which was treated successfully with radiotherapy again. Eight patients with diffused lesions were treated with chemotherapy (CHOP or CHOP-like courses). After initial treatment 5 (63%) of 8 patients had developed new skin lesions, and 2 of them had developed extracutaneous localizations. Then the 3 of the relapsed patients were treated with autologous stem cell transplantation, all of whom obtained complete remission. At the time of last follow-up (median follow-up: 34 months; range: 10–60 months), 5 patients are in complete remission, 4 patients have ongoing skin disease, while 3 patients have died, 2 of the complications of HPS or therapy-related side effects and 1 of unrelated disease. The 3-year OS and DSS of the patients were 75% and 42%, respectively. Patients without HPS had a significantly better 3-year OS (88%) than patients with HPS (50%; P <.001). Conclusion:SPTCL seems to be a kind of heterogeneity disease. The factors associated with an unfavorable disease course were: diffuse lesions, a low white blood cell count, elevated lactate dehydrogenase, and combine with HPS. Autologous stem cell transplantation may improve the overall survival of high risk patients. Disclosures:No relevant conflicts of interest to declare.

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of Three Cases.

Abstract 4703The World Health Organization (WHO) recently published a revised, updated edition of the WHO Classification of Tumours of the Haematopoietic and Lymphoid Tissues, including new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities – some defined mainly by genetic and immunophenotypic features – that have only recently been characterized. Particularly, the diagnosis and classification of acute leukemias of ambiguous lineage is debated; among these: “blastic NK-cell lymphoma” and “agranular CD4+/CD56+ hematodermic neoplasms”. Both of them are now known to be, in virtually all cases, a tumor derived from precursors of a specialized subset of dendritic cells, plasmacytoid dendritic cells, and so are myeloid-related neoplasms defined as blastic plasmacytoid dendritic cell neoplasm (BPDCN). This is a clinically aggressive neoplasm that is usually characterized at onset by solitary or multiple skin lesions, often with associated regional lymphadenopathy, and frequently by involvement of the PB and BM. Leukemic cells show submembranous cytoplasmic vacuoles and pseudopodia-like extensions of agranular cytoplasm. The blasts in such cases do not express myeloperoxidase or nonspecific esterase, and are characterized by the expression of CD4, CD43, CD56, CD123, BDCA-2/CD303, TCL1, and CLA; CD7 and CD33 are not uncommonly expressed as well, and TdT is expressed in about 30% of cases. There is no expression of CD34 or CD117. Here we report three cases with clinical data, cytological and immunophenotypic findings strongly suggesting the diagnosis of BPDCN. Case 1An 80 year-old-man was admitted to our institution on December 2006. He referred the occurrence of skin lesions since January 2005, when a diagnosis of extranodal B-cell non-Hodgkin lymphoma was made and treatment with conventional chemotherapy was performed, but without achieving any response. At our evaluation he presented leukocytosis (144 × 109/L) associated with purplish, firm nodules on the trunk, arms and face. Peripheral blood and bone marrow aspirate showed the presence of blast cells with a lymphoid appearance, granular periodic acid-Schiff (PAS) positivity and a high expression of CD33, CD4, and CD56. He was treated with AML-like therapy, but died of disease progression. Case 2A 79-year old woman was admitted in December 2006 with a 2-month history of anemia, splenomegaly, and weight loss of 10 kg in the last year. Laboratory tests were as follows: Hb, 41 g/L; leukocytes, 2.5 × 109/L (with 10% of blast cells); platelets, 43 × 109/L. No lymphadenopathy or skin lesions were present. Bone marrow examination revealed 41% of small to medium-sized blast cells without Auer rods or granula and negative reactivity to myeloperoxidase, esterase and PAS. She was treated with an AML-like protocol; she achieved partial response, but died after three months, of disease progression. Case 3A 69-year-old man was admitted to our Institution for cytopenia in June 2009. He referred the occurrence of brownish-purple firm nodules on the trunk since April 2009. At our evaluation he presented pancytopenia; bone marrow aspiration was performed and revealed infiltration by 65% of blasts with reticulated chromatin, evident nucleoli, a vacuolated cytoplasm and pseudopodia-like expansions. The blasts were negative for myeloperoxidase, monocyte esterase and PAS staining. Skin biopsy revealed a dermal infiltration by the same blastic-cell BM population. He underwent AML-like therapy and, although the skin lesions disappeared, 30% blastic bone marrow infiltration persisted.Morphological revision of these cases, selected for their peculiar immunophenotype reported in the following Table, revealed the same cytological features and cytochemical reactivity in cases 2 and 3; case 1 had a lymphoblastic-like morphology and showed PAS positivity, but the lack of cCD3 was not consistent with the diagnosis of ALL. All the cases were FLT3-ITD+. We suggest that a correct modern panel of MoAb with a careful morphological examination could help to pose the diagnosis of BPDCN, which typically affects older patients and is characterized by poor prognosis.CaseCD4CD56HLA-DR7.1cMPOcCD3cCD79aCD34CD117CD33CD3031++++-----+nt2++++-----++3++++-----++ Disclosures:No relevant conflicts of interest to declare.

The significance of reflectance confocal microscopy in the assessment of solitary pink skin lesions

Solitary pink lesions often manifest nondescript clinical and dermatoscopic primary morphologic features. The differential diagnosis for pink lesions tends, therefore, to be broad, ranging from inflammatory processes to malignancy. In vivo reflectance confocal microscopy (RCM) may help in the evaluation of pink lesions. We sought to demonstrate the use of RCM as an adjunct to the bedside diagnosis of pink lesions. We describe a series of patients with clinically and dermatoscopically equivocal pink lesions for which RCM examination allowed for a rapid and accurate diagnosis. All lesions were excised for histopathologic evaluation. Integrating the findings in the case series with a literature review, we present RCM diagnostic criteria for pink lesions. Lesions included basal cell carcinoma, squamous cell carcinoma, amelanotic melanoma, and inflamed seborrheic keratosis. RCM shows distinctive findings for each diagnostic entity when stratified by anatomic level into suprabasal epidermis, dermoepidermal junction, and papillary dermis. In the cases presented RCM allowed for a rapid and accurate noninvasive diagnosis. The study is descriptive and does not test accuracy of RCM criteria in a prospective series of pink lesions. RCM may add useful diagnostic features to the clinical evaluation of solitary pink lesions.