A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy

Jacob, Rojymon; Scala, Matthew; Fung, Maxwell A.

doi:10.1016/j.jaad.2008.05.042

Cited by 12 publications

(6 citation statements)

References 9 publications

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“…One illustration of this is that early stage FMF carries a much worse outcome than that of even late-stage classic MF or STMF, even when there is clinical and histopathologic overlap between these adnexal variants. 3,6 Molecular studies demonstrate a high concentration of CD1a cells localized to the infundibulum and isthmus exceeding a 10:1 ratio in 70% of lesional hair follicles of FMF analyzed, 19 whereas this is not so for STMF even in instances of brisk follicular involvement indicating an intrinsic difference between the two entities. 4 This variation in T-cell subtypes may account for the significant divergence clinical behavior.…”

Section: Discussionmentioning

confidence: 99%

“…Local radiotherapy, 19 systemic alitretinoin, 6 combination vorinostat and retinoids, 15 VELP (vincristine sulfate, etoposide, L-asparaginase, and prednisone acetate) chemotherapy, 11 and psoralen and UVA 6 have all been used to successfully treat STMF. Resistance to skin directed therapies mirrors FMF which, in the rare instance it responds to treatment, requires systemic agents.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Syringotropic mycosis fungoides (STMF) is a rare form of MF, first identified by Sarkany in 1969. 3–4, 7–19 It is characterized histopathologically by hyperplastic eccrine glands and ducts surrounded and permeated by a dense infiltrate of atypical lymphocytes in the deep dermis. 4 Although the clinical presentation of STMF is similar to folliculotropic mycosis fungoides (FMF), STMF is much less aggressive.…”

Section: Introductionmentioning

confidence: 99%

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Syringotropic Mycosis Fungoides: A Rare Form of Cutaneous T-cell Lymphoma Enabling a Histopathologic “Sigh of Relief”

Lehmer¹,

Amber

Feraudy

2017

The American Journal of Dermatopathology

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Syringotropic mycosis fungoides (STMF) is a very rare variant of cutaneous T-cell lymphoma (CTCL). It follows a much milder disease course than its clinically indistinguishable adnexal counterpart, folliculotropic mycosis fungoides (FMF). We report a case of a 36-year-old male who presented with erythematous, studded papules and plaques on the left upper extremity and right anterior thigh diagnosed as MF Stage 1A on initial superficial shave biopsy. Lesions recurred after initial improvement with narrow band ultraviolet light therapy demonstrating a concentration of abnormal lymphocytes around eccrine sweat glands on repeat biopsy, consistent with STMF. While the deeper, periadnexal infiltrate found in both STMF and FMF confers increased resistance to skin-directed therapies effective in classic MF, these entities diverge with respect to their clinical behavior. Syringotropism is a marker for increased disease specific survival whereas even FMF carries a prognosis worse than conventional MF. Increased awareness amongst the dermatopathology community of the histopathologic distinction between STMF and FMF is essential to guide treatment type, duration, and intensity in adnexal disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Syringotropic Mycosis Fungoides: A Rare Form of Cutaneous T-cell Lymphoma Enabling a Histopathologic “Sigh of Relief”

Lehmer¹,

Amber

Feraudy

2017

The American Journal of Dermatopathology

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“…Folliculotropic MF is considered to be a closely related condition, as some cases of syringotropic MF demonstrate lymphocytic infiltration involving both eccrine glands and hair follicles (23,24). On the other hand, lymphoid infiltrates around the eccrine glands without syringometaplasia can be observed in biopsies of conventional MF usually in the tumour stage (22,25,26).…”

Section: Discussionmentioning

confidence: 99%

A Case of Rapidly Progressive, Fatal Mycosis Fungoides Presenting as a Haematoma-like Lesion

Hattori¹,

Uchiyama²,

Tago³

et al. 2013

Acta Derm Venerol

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Mycosis fungoides usually follows an indolent clinical course. We report here a rapidly progressive case of mycosis fungoides with peculiar clinical and histological features, presenting as a haematoma-like mass on the thigh accompanied by multiple reddish brown erythematous lesions on the trunk and extremities. Histopathologically, the erythematous lesions showed epidermotropism of atypical T lymphocytes expressing CD4 and prominent syringotropism without syringometaplasia. The haematoma-like lesion consisted of diffuse and dense infiltrates of medium-to-large-sized pleomorphic lymphocytic cells expressing CD30, suggesting that CD30+ large-cell transformation had occurred. Mycosis fungoides presenting as a haematoma-like lesion is rare and may be a poor prognostic sign.

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“…Therefore, plantar MF is less responsive to skin-directed therapy, compared to other areas of the cutaneous with MF5. Mycosis fungoides and other variants of cutaneous T-cell lymphomas are very radiosensitive; local thick plaque skin lesions can be treated successfully with local radiotherapy6-10.…”

Section: Discussionmentioning

confidence: 99%

The Three Dimensional Conformal Radiotherapy for Hyperkeratotic Plantar Mycosis Fungoides

et al. 2011

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The localized early-stage of Mycosis fungoides (MF) (stage IA-IIA) is usually treated with topical agents, such as nitrogen mustard, steroids, and phototherapy (UVB/PUVA) as first line therapy; response to these initial treatments is usually good. However, hyperkeratotic plantar lesions are clinically rare and have decreased responsiveness to topical agents. For such cases, physicians may consider local radiotherapy. Here, a case of an 18-year-old Korean woman who was treated with three-dimensional conformal radiotherapy (3D-CRT) for hyperkeratotic plantar lesions that were refractory to UVA-1, methotrexate, and topical steroids is reported. Complete remission was attained after radiotherapy. During the one-year follow-up period, there has been no evidence of disease recurrence and no chronic complications have been observed.

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A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy

Cited by 12 publications

References 9 publications

Syringotropic Mycosis Fungoides: A Rare Form of Cutaneous T-cell Lymphoma Enabling a Histopathologic “Sigh of Relief”

Syringotropic Mycosis Fungoides: A Rare Form of Cutaneous T-cell Lymphoma Enabling a Histopathologic “Sigh of Relief”

A Case of Rapidly Progressive, Fatal Mycosis Fungoides Presenting as a Haematoma-like Lesion

The Three Dimensional Conformal Radiotherapy for Hyperkeratotic Plantar Mycosis Fungoides

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