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2.1
Primary Cutaneous Follicle Center Lymphoma
PCFCL

Presentation

Features contributing to the diagnostic conundrum in this case included an atypical clinical and histological presentation, lack of pertinent clinical history and multiple presentations at different institutions. [ncbi.nlm.nih.gov]

Entire Body System

  • Fishing

    Morphologic, immunohistochemical, conventional cytogenetic, and fluorescence in situ hybridization (FISH) studies were performed on this patient. [ncbi.nlm.nih.gov]

    Help me code for diagnosis year : Alternate Names Crosti's disease PCFCL PCFCL, leg type Reticulohistiocytoma of the dorsum Definitive Diagnostic Methods FISH Genetic testing Histologic confirmation Immunophenotyping Polymerase chain reaction (PCR) Genetics [seer.cancer.gov]

    Trisomy 3 was performed by fluorescent in situ hybridization (FISH) in 13 cases. Follow up was obtained in 17 cases (range 3 to 137 months). [nature.com]

    However, it can be observed by FISH in 8.7% of cutaneous cases and seems to be associated with extra-cutaneous disease. [oskar-bordeaux.fr]

Musculoskeletal

  • Long Arm

    We report a reciprocal translocation between the long arms of chromosomes 12 and 21, t(12;21)(q13;q22), in a patient with primary cutaneous follicle center lymphoma. [ncbi.nlm.nih.gov]

    The other interesting finding in this case is the deletion present on the long arm of one chromosome 10. [path.upmc.edu]

    Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. The disease likely begins as a hyperreactive inflammatory process. [emedicine.medscape.com]

Skin

  • Subcutaneous Nodule

    A 62-year-old man presented with a solitary indolent subcutaneous nodule for 3 years duration, without other abnormalities. [ncbi.nlm.nih.gov]

    Whole-body Positron Emission Tomography--Computed Tomography scan revealed a 1.9 cm subcutaneous nodule in the anteromedial soft tissues of the left thigh with marked increased radiotracer uptake. [ijdvl.com]

    Cases with a γ/δ T- cell phenotype are seperately classified as cutaneous γ/δ T-cell lymphomas. 60.  Clinical Features :  Multiple subcutaneous nodules involving trunk and extremities, rarely, the face.  Constitutional symptoms : fever, fatigue, and [slideshare.net]

  • Partial Alopecia

    Partly hypopigmented, infiltrated lesion on the temporal area with partial alopecia. Biopsy revealed specific infiltrates (“alopecia neoplastica”). Figure 11.5 Cutaneous follicle center lymphoma. Agminated erythematous papules on the eyebrow. [plasticsurgerykey.com]

Workup

Follow-up Patients should be seen in an outpatient setting at least every 6 months for a clinical workup. They do not require any additional laboratory workup unless enlargement of the regional lymph nodes has occurred. [emedicine.medscape.com]

For further workup, a 6 mm punch biopsy was done from the lesion. Histologically, the skin biopsy showed an unremarkable epidermis. [ijpmonline.org]

Hence, all patients with ocular lymphoma should have a complete workup to rule out systemic lymphoma. " ________________________ Intra-ocular lymphoma: A rare malignant form of eye cancer. [lymphomation.org]

Treatment

In conclusion, intravenous rituximab therapy is a safe and effective treatment for multifocal PCFCL and may represent an excellent alternative treatment to radiotherapy in multifocal PCFCL. [medicaljournals.se]

Due to a refusal to treatment, the lesion progressively grew until now. [ncbi.nlm.nih.gov]

In patients who do require some treatment intervention, complete surgical excision of the lesion or lesions in question is common practice, as is radiotherapy. [lymphomainfo.net]

Skin-directed treatments: For early stages of MF, treatments are aimed at the skin. [cancer.org]

Prognosis

The distinction between primary and secondary B-cell lymphomas is essential for defining prognosis/course of action. [ncbi.nlm.nih.gov]

The relapse rate is about 30 percent, but often relapse does not correlate with prognosis. Where prognosis gets a little tricky is on the legs. [lymphomainfo.net]

Recurrence is common following treatment (60%+) but does not imply a worse prognosis. [dermcoll.edu.au]

The diffuse pattern may resemble diffuse large B-cell lymphoma, but the prognosis remains excellent, and the diagnosis should still be follicle center lymphoma. The neoplastic lymphocytes stain for pan-B-cell markers and usually for bcl-6. [dermpedia.org]

Etiology

[…] with the presence of bcl-6 and occasional network of follicular dendritic cells in the background. [4] An association with Borrelia burgdorferi, hepatitis C, or human herpesvirus 8 has been described in sporadic patients but does not seem to be a major etiological [bioline.org.br]

The etiology may involve microbial, physical, chemical, Borrelia burgdorferi infection, insect bite, tattoo, and drugs Clinically, several variants of C-PSL exist. .... [lymphomation.org]

Association with infections such as Borrelia burgdorferi, hepatitis C, or human herpesvirus 8 (HHV-8) has been described in sporadic patients, but does not seem to be an important etiologic factor for cutaneous follicle center lymphoma [15, 16]. [plasticsurgerykey.com]

(Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. [dovemed.com]

Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. The disease likely begins as a hyperreactive inflammatory process. [emedicine.medscape.com]

Epidemiology

Immunohistochemistry Immunophenotyping Progression and Transformation Cutaneous relapses noted in ~30% of patients Recurrences usually proximate to the initial site of the tumor Untreated skin lesion s increase in size, rarely metastases to other site s Epidemiology [seer.cancer.gov]

B-cell lymphoma of follicle center cells, primary in skin Disease limited to skin for 6 months after diagnosis Distinct disease from systemic follicular lymphoma Better prognosis Fewer BCL2 rearrangements than systemic follicular lymphoma CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

WHO Classification Most common histology: diffuse large B-cell lymphoma EORTC Classification Note: in the EORTC, diffuse large B-cell lymphomas of non-leg site are classified as follicle center cell (i.e., considered indolent instead of aggressive) Epidemiology [en.wikibooks.org]

Epidemiology : occurs mostly in adults, but may occasionally affect children and adolescents. [ufrgs.br]

Peñate Y, Hernández-Machín B, Pérez-Méndez LI, Santiago F, Rosales B, et al. (2012) Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicentre study. [omicsonline.org]

Pathophysiology

Pathophysiology The development of cutaneous lymphoma is only partially understood. Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. [emedicine.medscape.com]

Prevention

Skin-directed therapies Topical chemotherapy – creams containing drugs which may be used to kill and prevent the growth and division of cancer cells Phototherapy UVB – broad and narrow band, and PUVA – the use of ultraviolet light to treat the skin Radiation [leukaemia.org.au]

How can Primary Cutaneous Follicular Center Lymphoma be Prevented? Presently, the cause of Primary Cutaneous Follicular Center Lymphoma is unknown and there are no known methods to prevent its formation. [dovemed.com]

IRB 14321: A Single-Arm, Open-Label, Multicenter, Study of Folotyn® (Pralatrexate Injection) in Combination with Oral Leucovorin to Prevent or Reduce Mucositis in Patients with Relapsed or Refractory (R/R) Peripheral T-Cell Lymphoma (PTCL). [cityofhope.org]

Microdissection was performed as follows: 4-μm sections cut from formalin-fixed, paraffin-embedded tissues were mounted on glass slides with a thickness of 0.17 mm (very thin glass slides are needed to prevent laser energy from being dispersed before [bloodjournal.org]

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