Additional Findings of 18 F-AIF-FAPI-42 PET/CT in a Patient With Mycosis Fungoides-Type Cutaneous T-Cell Lymphoma : Comparisons With 18 F-FDG PET/CT.

ABSTRACT: A 44-year-old woman presented with extensive skin patches and pruritus persisting for 3 years. Histopathological examination of the skin from the right abdomen confirmed mycosis fungoides-type cutaneous T-cell lymphoma. Staging PET with 18 F-FDG PET/CT) showed increased uptake in the skin on the right abdomen and left hip. Subsequently 18 F-FAPI-42 PET/CT revealed additional foci of abnormal uptake on the skin of the chest and back.

Extensive Cutaneous-Mucosal and Muscular Involvement of Gamma/Delta Cutaneous T-Cell Lymphoma on 18 F-FDG PET/CT.

ABSTRACT: Gamma/delta T-cell lymphoma is a rare and aggressive subtype of primary cutaneous lymphoma. Clinical manifestations typically include the development of subcutaneous nodules and ulcerated plaques. Some forms present as panniculitis with hemophagocytic syndrome. Prognosis is bleak, with a 10% 5-year survival rate. In this report, we present the case of a 20-year-old man from French Polynesia, referred for 18 F-FDG PET/CT because of the progressive worsening of febrile cutaneous-mucosal infiltration on the face persisting for 1 month. PET examination guided a biopsy from the right deltoid muscle, and expert histological analysis confirmed a CD8 + not otherwise specified T-cell lymphoma, granzyme+ and TCR gamma/delta.

Non-invasive Skin Imaging in Cutaneous Lymphomas.

The diagnosis of cutaneous lymphomas is challenging and requires skin tissue for histology and immunophenotyping using immunohistochemistry and molecular studies. In recent years, the role of non-invasive imaging techniques has been described as part of the clinical assessment of cutaneous lymphoma lesions. Imaging modalities such as dermoscopy, reflectance confocal microscopy (RCM), and high frequency ultrasound (HFUS) have been shown to be very valuable in raising the clinical suspicion for lymphomas of the skin, and in distinguishing cutaneous lymphomas from inflammatory dermatoses such as lupus, psoriasis, or eczema. These non-invasive methods can be used to direct the clinician to the optimal biopsy site to maximize the histopathological results and minimize false negatives. These methods also have a potential place in monitoring treatment response. In this review we present a concise summary of the dermoscopic imaging, RCM, and HFUS features seen in cutaneous T-cell lymphomas (CTCL) and B-cell lymphomas (CBCL).

Cutaneous T-cell lymphoma in erythrodermic cases may be suspected on the basis of scalp examination with dermoscopy.

Erythrodermic variants of cutaneous T-cell lymphoma (CTLC) are one of the case of erythroderma. The aim of the study was to assess the value of scalp dermoscopy in differentiation between erythrodermic CTCL, psoriasis, and atopic dermatitis. A total of 76 patients were included into the study (16 patients with erythrodermic CTCL, 20 patients with psoriatic erythroderma, 20 with erythrodermic atopic dermatitis, and 20 healthy volunteers). The most common trichoscopic features of erythrodermic CTCL were: numerous pili torti, numerous broken hairs, white thick interfollicular bands, and patchy hyperpigmentation of the background. They were observed in 81% (13/16), 75% (12/16), 56% (9/16), and 37.5% (6/16) of patients with CTCL, respectively (p < 0.001). Other specific features of erythrodermic CTCL were 8-shaped hairs (19%; 3/16) and visible anagen bulbs (12.5%; 2/16) (p < 0.05 and p = 0.052, respectively). The most common vascular pattern of erythrodermic CTCL was perifollicular arrangement of glomerular (50%; 8/16; p < 0.001) or linear vessels (31%; 5/16; p < 0.05). Follicular spicules-like scaling was pathognomonic for erythrodermic CTCL (12%, 2/16) although its presence did not reach statistical significance (p = 0.052). In conclusion, the characteristic trichoscopic findings of erythrodermic CTCL are numerous pili torti, eight-shaped hairs, thick white interfollicular bands, color heterogeneity of the background and perifollicular arrangement of vessels.

Primary Cutaneous Natural Killer/T-Cell Lymphoma: A Posttransplant Lymphoproliferative Disorder Demonstrated by 18F-FDG PET/CT.

ABSTRACT: A 24-year-old man with a history of hemophagocytic lymphohistiocytosis (HLH) presented with swelling of the left foot and skin ulcer. The patient received bone marrow transplantation for HLH 3 years ago for his HLH. Biopsy of left foot skin demonstrated primary cutaneous natural killer/T-cell lymphoma: a posttransplant lymphoproliferative disorder. FDG PET/CT images demonstrated multiple foci of abnormal accumulation in the body, especially in the skin. Follow-up PET/CT after chemotherapy demonstrated that most abnormal activities disappeared except for the lesion in the left foot.

Primary Cutaneous Acral CD8+ T-Cell Lymphoma-A Single Center Review of 3 Cases and Recent Literature Review.

The recently published 2016 revision of the WHO classification of lymphoid neoplasms includes primary cutaneous acral CD8-positive T-cell lymphoma (PCATCL) as a provisional entity. This is a rare indolent lymphoma characterized by papules or nodules on the ear and a dermal infiltrate of CD8-positive T-lymphocytes with cytotoxic marker expression. A retrospective review of a single institutional experience with PCATCL identified 3 patients (mean age 54; range 49-62) with papules or nodules on the ear. Lesional biopsies demonstrated a dense diffuse dermal infiltrate of atypical lymphocytes with a Grenz zone in 2 cases and focal epidermotropism in 1 case. The atypical lymphocytes were predominantly CD3 and CD8 positive with expression of cytotoxic marker TIA1. Staging evaluation failed to reveal systemic disease. Two patients underwent local excision, and the third received local radiation therapy all with complete response and no disease recurrence at last follow-up 3 months (range 2-5 months). Our cases add to the existing limited literature on the clinical and histopathological features of PCATCL. We also performed an updated systematic literature view of the entity.

Small Primary Cutaneous γδT-Cell Lymphoma Lesions Successfully Treated With Pralatrexate.

Here we report on the treatment of a 38-year-old woman with primary cutaneous γδT-cell lymphoma, which is a rare subset of cutaneous T-cell lymphoma. She presented with multiple subtle subcutaneous nodules, which were not clearly observed on computed tomography scans or after biopsy. However, F-fluorodeoxyglucose positron emission tomography (F-FDG-PET) accurately detected small cutaneous lesions. She achieved a second complete remission, as demonstrated by F-FDG-PET performed after pralatrexate infusion.

Dermoscopy of Lymphomas and Pseudolymphomas.

Primary cutaneous lymphomas are a heterogeneous group that includes 2 main groups of primary T- and B-cell lymphomas, which can involve the skin with distinct variability in clinical presentation, histopathology, immunophenotypes, molecular signature, and prognosis. The authors describe the most frequent clinical forms of cutaneous lymphomas and their dermoscopic features. Even if the diagnosis of these entities is still based on a cellular level and the literature on dermoscopy in cutaneous lymphomas is limited and, for several entities it is based only on single case reports/case series, we think that know how they appear also in dermoscopy can be useful for helping in the clinical diagnosis.

Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. OBJECTIVE: To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. METHODS: A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. RESULTS: The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%. LIMITATIONS: This was a retrospective study. CONCLUSIONS: SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.

Lymphoma Involving Skin and Subcutaneous Fat of the Breast: Two Cases.

Breast lymphoma is uncommon, and it usually involves breast parenchyma and ipsilateral axillary lymph nodes. We report rare cases of cutaneous T-cell lymphoma involving the skin and subcutaneous fat layers mimicking panniculitis of the breasts in a 33-year-old male patient who presented with a growing palpable mass and pain in both breasts and in 17-year-old female patient who presented with an indolent palpable breast mass for 1 year. Extranodal natural killer/T-cell nasal-type and subcutaneous panniculitis-like T-cell lymphomas were diagnosed after skin punch biopsies. Here we report the clinical manifestations, mammographic and ultrasound findings, and the difficulties in histological diagnosis of the cases.

A rare localised nasal CD30+ primary cutaneous T-cell lymphoma following liver transplantation.

Cutaneous T-cell post-transplant lymphoproliferative disorder (PTLD) is a rare clinical presentation that can potentially turn aggressive in solid-organ transplant recipients if not detected and intervened on early. We encountered a rare case of rapidly worsening primary cutaneous CD30-positive, Epstein-Barr virus-negative anaplastic large cell lymphoma (ALCL) of T-cell origin, manifesting as an isolated nasal tip lesion in a 71-year-old man 4 years after orthotopic liver transplantation. Excisional biopsy with partial rhinectomy showed subepithelial diffuse infiltration of medium-to-large lymphoid cells having round-to-irregular nuclei, partially condensed chromatin and prominent nucleoli. Immunophenotypic studies revealed CD30-positive primary cutaneous ALCL. Positron emission tomography/CT imaging revealed a locally active disease, and radiation therapy was initiated with complete response. A high index of suspicion for PTLD when evaluating skin lesions in a post-transplant patient is paramount for its early recognition, prompt diagnosis and timely intervention while the window for curative therapy remains possible.

T-Cell Non-Hodgkin Lymphomas: Spectrum of Disease and the Role of Imaging in the Management of Common Subtypes.

T-cell non-Hodgkin lymphomas (NHLs) are biologically diverse, uncommon malignancies characterized by a spectrum of imaging findings according to subtype. The purpose of this review is to describe the common subtypes of T-cell NHL, highlight important differences between cutaneous, various peripheral and precursor subtypes, and summarize imaging features and the role of imaging in the management of this diverse set of diseases.

The utility of positron emission tomography with and without computed tomography in patients with nonmelanoma skin cancer.

Combined positron emission tomography (PET) and computed tomography (CT) scans are widely used in the staging and monitoring of most malignancies. The differential for PET-positive cutaneous lesions includes primary skin cancers, infections, cutaneous metastases from distant malignancies, and benign neoplasms. In dermatology, PET/CT scans have been most widely studied in patients with melanoma and Merkel cell carcinoma. The role of PET/CT scans in the management of other cutaneous malignancies is less clear, but it has shown great promise in the management of patients with squamous cell carcinoma and cutaneous lymphoma. This review seeks to address the usefulness of PET/CT scans in nonmelanoma skin cancer and to provide guidance regarding the management of patients with incidental PET-positive nodules. Currently, there is limited experience with PET/CT scans for staging and monitoring of non-head and neck metastatic basal cell and squamous cell carcinomas, and results show limited sensitivity and specificity. We also address the evidence for management of an incidental PET-positive cutaneous nodule and recommend obtaining a biopsy specimen in patients with a known noncutaneous malignancy, a history of primary skin cancer, or a high risk of either cutaneous or noncutaneous malignancy.

Hematopoietic stem cell transplantation for primary cutaneous γδ T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma.

BACKGROUND: The panniculitic T-cell lymphomas (TCLs) comprise 2 distinct entities, αß subcutaneous panniculitis-like TCL (SPTCL) and the γδ cutaneous TCLs with pannicular involvement primary cutaneous γδ (PCGD)-TCL. Although outcomes for most patients with SPTCL are favorable, those with PCGD-TCLs generally have an inferior outcome, and treatment strategies have not been well defined. Allogeneic hematopoietic stem cell transplantation (HSCT) has been shown to be a potentially curative strategy in aggressive TCLs and in refractory and advanced-stage mycosis fungoides. OBJECTIVE: We sought to analyze the outcomes of HSCT for panniculitic cutaneous TCL. RESULTS: Fourteen patients (4 SPTCL, 10 PCGD-TCL) presented with primarily pannicular T-cell infiltrates. Seven patients underwent allogeneic HSCT from matched-related donors and matched-unrelated donors of which 4 (57%) are alive (1 SPTCL, 3 PCGD-TCL) at 7.8, 6.9, 6.2, and 0.25 years. Two patients underwent autologous HSCT (1 SPTCL, 1 PCGD-TCL) and both are alive at a median follow-up of 1.91 years. LIMITATIONS: This study is limited by its retrospective nature and small sample size because of the rarity of SPTCL and PCGD-TCL. CONCLUSION: Aggressive therapy followed by allogeneic HSCT is a promising treatment modality for patients with PCGD-TCL.

Primary cutaneous CD8+ cytotoxic T-cell lymphoma involving the epidermis and subcutis in a young child.

CD8+ cytotoxic T-cell lymphoma involving the skin represents a heterogeneous group of diseases that include subcutaneous panniculitis-like T-cell lymphoma, primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, and 'type D' lymphomatoid papulosis. In this report, we describe a case of CD8+ cytotoxic T-cell lymphoma involving both the epidermis and subcutis. The patient was a 6-year-old girl who presented with a 3-year history of multiple plaques on her trunk and legs. The lesions had relapsed twice but responded well to prednisone. Histopathologic examination showed the proliferation of atypical lymphocytes in the epidermis, dermis and subcutaneous tissue. On immunohistochemical analysis, the atypical lymphocytes were positive for ßF1, CD3, CD8, perforin, granzyme B and TIA-1, but negative for T-cell receptor (TCR) γ, CD4, CD30 and CD56. It was difficult to classify this tumor in terms of the known types of cutaneous lymphoma, and this case should be differentiated with subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma.