Subcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology

2020 ◽  
Vol 9 (1) ◽  
pp. 120-123
Author(s):  
Debasis Gochhait ◽  
Shailesh Kekade ◽  
Durga Devi ◽  
Bheemanathi Hanuman Srinivas ◽  
Neelaiah Siddaraju ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma

Sinonasal T-cell Lymphoma and Wegener's Granulomatosis: Aspects in Early Differential Diagnosis

1996 ◽  
Vol 10 (4) ◽  
pp. 239-246
Author(s):  
Anders Cervin ◽  
Michael Dictor ◽  
Olof Kalm
Keyword(s):  
Differential Diagnosis ◽  
In Situ Hybridization ◽  
T Cell ◽  
Wegener’S Granulomatosis ◽  
Cell Lymphoma ◽  
Wegener's Granulomatosis ◽  
T Cell Lymphoma ◽  
Upper Airways ◽  
Gastrointestinal Complications

The clinical course of 12 patients with sinonasal T-cell lymphoma retrospectively diagnosed using in situ hybridization for Epstein-Barr virus RNA was compared with that of 10 recently treated patients with Wegener's granulomatosis (WG) in the upper airways. In particular, we studied the presenting signs and symptoms of both diseases, which commonly offer a problem in differential diagnosis at the clinical and pathological level. A bimodal age distribution was suggested in both T-cell lymphoma and WG; five patients with T-cell lymphoma developed disease prior to 40 years of age. Four of the 12 lymphoma patients had a history of “chronic rhinitis” for several years before developing mucosal ulcerations, which were initially unilateral, as opposed to the bilateral ulcerations in early sinonasal WG. Two lymphoma patients had swelling of the nasal dorsum and cheek. In contrast to the WG patients, cases of T-cell lymphoma did not exhibit associated clinical signs of arthritis, conjunctivitis, pulmonary lesions, or nephritis in the early stage of the disease. Nine of the patients with T-cell lymphoma presenting as a sinonasal lesion developed disseminated disease, variably including infiltrates in intestine, lung, CNS, and skin. Four of these patients died from gastrointestinal complications of their disease. We conclude that unilateral ulcerative or hemorrhagic polypoid mucosal lesions in the sinonasal area are suggestive of lymphoma rather than WG, and nonspecific symptoms, at least in Western patients, may be present as early as the second or third decade of life. A biopsy specimen containing T lymphocytes positive for the EBV ribonucleoprotein EBER1 on in situ hybridization offers reliable confirmation of T-cell lymphoma and is of differential diagnostic value against WG.

A Young Man with Fever and Skin Nodules

2008 ◽  
Vol 12 (6) ◽  
pp. 299-301
Author(s):  
Mark A. Lomaga ◽  
Scott Walsh
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Clinical Presentation ◽  
Rare Variants ◽  
Recent Literature ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Subcutaneous Nodules ◽  
Skin Nodules

Background: There are two rare variants of cutaneous T-cell lymphoma (CTCL) that primarily involve the subcutis. These include subcutaneous panniculitis-like T-cell lymphoma (SPTL) and cutaneous γ/δ T-cell lymphoma. Objective: This case report describes the clinical presentation, diagnosis, and treatment of a young man with probable SPTL. A review of recent literature outlining the differences between SPTL and cutaneous γ/δ T-cell lymphoma is discussed. Conclusion: The differential diagnosis in patients presenting with subcutaneous nodules and constitutional symptoms should include CTCL.

Molecular Diagnosis of Peripheral T-Cell Lymphoma/NOS From Formalin Fixed Paraffin Embedded Tissues,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3662-3662
Author(s):  
Pier Paolo Piccaluga ◽  
Antonio De Leo ◽  
Maura Rossi ◽  
Maria Antonella Laginestra ◽  
maria Rosaria Sapienza ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Conflicts Of Interest ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Whole Genome ◽  
Mrna Levels ◽  
Molecular Signatures ◽  
Test Set ◽  
Formalin Fixed

Abstract Abstract 3662 The differential diagnosis among the commonest peripheral T-cell lymphomas (PTCLs) (i.e. PTCL not otherwise specified, NOS; angioimmunoblastic T-cell lymphoma, AITL; and anaplastic large cell lymphoma, ALCL) is difficult, the morphologic and phenotypic features being largely overlapping. Noteworthy, recent international studies indicated significant differences in their clinical behavior as well as concerning the presence of potential therapeutic targets. We performed whole genome gene expression profiling (GEP) of PTCLs aiming to identify molecular signatures able to improve their diagnosis. We studied 95 PTCLs, including 73 PTCLs/NOS, 12 ALCLs (6 ALK+ and 6 ALK-), and 10 AITLs. All tissue samples were formalin-fixed and paraffin embedded (FFPE). GEP was performed by Illumina Whole Genome DASL Assay. First, we documented the efficiency of GEP from FFPE tissues by comparing the mRNA levels and the presence of the corresponding protein, including expressed (i.e. CD3) and not expressed (i.e. BCL10) molecules. Secondly, we tried to discriminate different PTCLs basing on their GEPs. By dividing a training (N=47) and a test set (N=48), we found 2 signatures able to differentiate PTCL/NOS vs. AITL and PTCL/NOS vs. ALCL ALK-. Specifically, in the test set the sensitivity (ST) and specificity (SP) of the assays were 100% – 80% (PTCL/NOS vs. AITL) and 100% – 100% (PTCL/NOS vs. ALK- ALCL) (Table 1). Accordingly, the positive (PPV) and negative (NPV) predicting values for the identification of PTCL/NOS were 0.92 and 1 (vs. AITL) and 1 and 1 (vs. ALK- ALCL) (Table 1).Table 1.Accuracy of GEP based signature in differentiating PTCL subtypesSTSPPPVNPVTraining setPTCL/NOS vs. AITL100%80%0.921PTCL/NOS vs. ALK-ALCL100%100%11Test setPTCL/NOS vs. AITL92.50%100%10.77PTCL/NOS vs. ALK-ALCL92.50%100%10.67Validation setPTCL/NOS vs. AITL85%86%0.920.76PTCL/NOS vs. ALK-ALCL96%73%0.960.73 Interestingly, the identified genes represented relevant functional pathways differentially regulated in the 3 tumour types, including protein kinase cascade, proliferation, and cell cycle. When applied to the test set of cases, the assay correctly classified 37/40 PTCLs/NOS (92.5%), 5/5 AITLs, and 3/3 ALK- ALCLs. Finally, we tested our signatures on 133 independent PTCL cases (including 78 PTCL/NOS, 43 AITL, and 12 ALK- ALCL) for which GEP data were available on the GEO database and were originally obtained from fresh/frozen tissues. Interestingly, we could efficiently recognize PTCL/NOS cases vs. AITLs (ST, 85%; SP 86%; PPV 0.92; NPV 0.76) and vs. ALK- ALCLs (ST 96%; SP 73%; PPV 0.96; NPV 0.73). In conclusion, we successfully generated for the first time GEP from routinary FFPE PTCL samples, identifying molecular signatures potentially useful for the clinical practice and, specifically, for the differential diagnosis of PTCL types. Disclosures: No relevant conflicts of interest to declare.

Primary Cutaneous Small to Medium-Size CD4+ Pleomorphic T-Cell Lymphoma: 48 Patients from Two Independent Centers and Revaluation of Clinico-Histological Criteria for Differential Diagnosis from Other CTCL

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2986-2986
Author(s):  
Silvia Alberti-Violetti ◽  
Rakhshandra Talpur ◽  
Carlos Antonio Torres-Cabala ◽  
Laura Corti ◽  
Emilio Berti ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Head And Neck ◽  
Mycosis Fungoides ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Histological Features ◽  
The Mean ◽  
Multifocal Disease

Abstract Introduction and Background: Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (PC-CD4+ SM-PTL) was recently re-classified as a rare provisional subtype in the 2008 World Health Organization (WHO) classification. It is characterized by solitary nodules or plaques located mostly on the head and neck. Multiple lesions are associated with a worse prognosis; however, the paucity of patients has prevented the definition of prognostic factors. Histologically, a dense cutaneous infiltrate of clonal small/medium hyperchromatic lymphocytes admixed with numerous reactive cells is observed, sometimes leading to the diagnosis of pseudolymphoma or other lymphoma: Mycosis Fungoides (MF) or peripheral T cell lymphoma – not otherwise specified (PTCL-NOS). Differential diagnosis with other aggressive cutaneous lymphomas is very important due to the favorable prognosis of PC-CD4+SM-PTL and complete response to conservative therapies. The purpose of this retrospective study was to evaluate and compare clinical and histological features of PC-CD4+ SM-PTL in 48 cases collected from two independent centers, MD Anderson Cancer Center (Houston, TX) and Dermatological Clinic of the University of Milan (Milan, Italy). Results: Forty-eight patients were identified (26 MDACC and 22 Milan cases). Women out-numbered men (women 67% and 55% respectively, and men 33% and 45% respectively). The mean age at onset was 53 and 50 years. The mean follow-up time was 19 months (1-84 months) for the American group and 31 months (3-180 months) for the Italian group. All patients in both groups were alive at the time of last follow up, without evidence of extra-cutaneous disease. Seventy-four percent of American patients and 73% of Italian patients developed single lesions, most common were nodules in the head and neck area. Multifocal lesions were found in other body sites besides head and neck in 6/7 American patients and in all Italian patients. Multifocal disease was characterized by plaques rather than nodules. Histological features showed a dense infiltrate composed of small to medium-sized hyperchromatic “grouped” atypical lymphoid cells occupying superficial and deep dermis. The infiltrate also showed admixed reactive B-cells, plasma cells, macrophages, and few dendritic cells. Vascular hyperplasia was noted. Adnexotropism was frequently found. Focal epidermotropism was also present. The neoplastic cells were CD3+, CD4+, CD5+, PD1+, CD8-, CD30-. A peculiar perivascular and small cluster arrangement of PD-1+ cells was noted. T-bet nuclear expression was recorded in 3 Italian cases. Mib1/Ki-67 proliferation index was between 5 and 30% in all Italian patients. The treatments used for patients with complete responses included surgical excision, local radiotherapy, and high potency topical steroids, occasionally combined with topical nitrogen mustard. Patients who had complete responses without relapse also had single lesions on the head and neck (67% and 68 % respectively), except for one US patient with multifocal disease. A small group of patients (18.5% and 4%, respectively) relapsed one time. Seven percent of American patients and 18% of Italian patients developed multifocal plaques on the trunk and extremities and had dramatic responses to therapy with a “waxing and waning” course. Only one Italian patient developed rapidly progressive tumors confined mainly to the legs and was resistant to cyclophosphamide and oral steroids. Conclusion: This case series demonstrates that PC-CD4+ SM-PTL is characterized by heterogeneous clinical presentations. There are no definite criteria to predict an aggressive course reported rarely in the literature. Histological findings useful, but not sufficient for diagnosis, and not sufficiently underscored by classical description of this tumor, are the arrangement of PD-1+ atypical cells in small clusters or rosettes surrounding reactive cells and the perivascular distribution of the neoplastic cells. Absent or minimal epidermotropism, a mixed B cell infiltrate and the clinical presentation of a solitary dermal nodule, may help to distinguish PC-CD4+ SM-PTL from the other CD4+ PD1+ CTCLs, mycosis fungoides and PTCL-NOS. Disclosures No relevant conflicts of interest to declare.

scholarly journals Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

2014 ◽  
Vol 138 (10) ◽  
pp. 1307-1318 ◽  
Author(s):  
Thanh T. Ha Lan ◽  
Noah A. Brown ◽  
Alexandra C. Hristov
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Lymphoid Hyperplasia ◽  
T Cell Lymphoma ◽  
Reactive Lymphoid Hyperplasia ◽  
Genetic Features ◽  
Patient Prognosis

Context.—Primary cutaneous CD4+ small/medium T-cell lymphoma is a provisional and controversial entity with a broad differential diagnosis. Despite being an uncommon lymphoma, it is a frequent diagnostic consideration in cutaneous biopsies with a dense lymphoid infiltrate because it shows overlapping features with reactive lymphoid hyperplasia (pseudolymphoma) and a variety of other primary cutaneous and systemic lymphomas. However, proper classification of this process is important for determining patient prognosis and treatment options. Objective.—To review the clinical, morphologic, immunophenotypic, and genetic features of primary cutaneous CD4+ small/medium T-cell lymphoma and contrast those features with entities in the differential diagnosis. Data Sources.—Applicable literature will be reviewed with emphasis on current controversies and distinguishing characteristics. Conclusions.—Although many consider primary cutaneous CD4+ small/medium T-cell lymphoma to be indistinguishable from reactive lymphoid hyperplasia/pseudolymphoma, it can be differentiated from other primary cutaneous and systemic lymphomas. Patients with solitary lesions of primary cutaneous CD4+ small/medium T-cell lymphoma generally have an excellent prognosis. Nevertheless, a subset of patients who have been reported to meet criteria for this lymphoma have followed a more-aggressive course; however, those patients show some differing clinical, morphologic, and immunophenotypic features.

scholarly journals T-Cell Lymphomas Presenting as Colon Ulcers and Eosinophilia

2015 ◽  
Vol 9 (2) ◽  
pp. 246-252 ◽  
Author(s):  
Ping-Hsiu Wu ◽  
Kuang-En Chu ◽  
Yu-Min Lin ◽  
Shu-Han Huang ◽  
Chin-Chu Wu
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Watery Diarrhea ◽  
Clinical Behavior ◽  
Biopsy Specimens ◽  
T Cell Lymphomas ◽  
Colon Biopsy ◽  
Primary Colon

Primary gastrointestinal T-cell lymphoma is an uncommon entity and primary colon T-cell lymphoma is even rarer. The majority of enteropathy-associated T-cell lymphomas present predominantly as ulcers or strictures in the endoscopic examinations, while primary B-cell lymphomas commonly present as exophytic lesions. Ulcerative colon T-cell lymphoma may mimic Crohn's disease (CD), which is a chronic inflammatory disease of the intestines with ulcer and fistula formations difficult for clinicians to diagnose based on endoscopic observations alone. Like CD, T-cell lymphoma may be characterized by the presence of multiple skipped ulcers distributed from the terminal ileum to the descending colon. Furthermore, it is difficult to diagnose this unusual lymphoma by a single endoscopic biopsy. Typically, the histological composition of T-cell lymphoma is made of medium to large atypical cells located in the base of the ulcer with extension to the muscle layer and the adjacent mucosa. However, it is common that biopsy specimens show only mixed inflammatory changes where the lymphoma cells are hard to be identified. The differential diagnosis of malignant lymphoma must be considered when clinically diagnosed CD is refractory to the medical treatment or when its clinical behavior becomes aggressive. The current study presents a rare case of primary colon T-cell lymphoma in a 56-year-old male with marked recent weight loss, watery diarrhea and bilateral neck lymphadenopathy, who received a laboratory checkup and endoscopic workup for colon biopsy. The initial pathological report was consistent with mucosal inflammation and benign colon ulcers. Interestingly, the blood test showed a prominent eosinophilia. A biopsy of the enlarged neck lymph nodes done approximately 1 month after the colon biopsy unexpectedly showed T-cell lymphoma, which led to a review of the initial colonic biopsy specimens. Additional immunohistochemical stains were used accordingly, which showed positive results for CD3, CD45RO and LCA antibodies confirming the diagnosis of lymphoma. The endoscopic diagnosis of ulcerative colon T-cell lymphoma is frequently confused with inflammatory conditions of the large bowel such as CD, and tuberculosis colitis. Our study aims to emphasize the difficulty in differentiating this ulcerative form of colon T-cell lymphoma from the inflammatory bowel diseases and the importance of its differential diagnosis due to the much more aggressive clinical behavior of the T-cell lymphoma.

Primary Cutaneous Peripheral T-Cell Lymphoma in a Sporotrichoid Pattern: A Case Report

2017 ◽  
Vol 21 (6) ◽  
pp. 568-571
Author(s):  
Daniel J. Lewis ◽  
Harina Vin ◽  
Tiffany Hinojosa ◽  
Michael T. Tetzlaff ◽  
Bouthaina S. Dabaja ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Radiation Therapy ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cutaneous Lymphoma ◽  
Atypical Presentation ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
History Of

We present the extraordinary case of a 72-year-old man with a history of primary cutaneous peripheral T-cell lymphoma not otherwise specified (pcPTCL-NOS) previously controlled with topical agents who developed tumours in a sporotrichoid pattern. Culture of the tumours was negative, and histopathology showed findings consistent with recurrent pcPTCL. The tumours were successfully treated with localised radiation therapy. Sporotrichoid lesions are an extremely rare and atypical presentation of cutaneous lymphoma, with only 2 other cases reported in the literature. Our case reinforces the need to include cutaneous lymphoma in the differential diagnosis of nodules on the extremities spreading in a sporotrichoid pattern. Clinical recognition of this atypical presentation of cutaneous lymphoma allows for prompt, effective treatment, which might include localised radiation therapy.

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