Primary Cutaneous Mantle Cell Lymphoma of the Leg With Blastoid Morphology and Aberrant Immunophenotype

Cesinaro, Anna Maria; Bettelli, Stefania; Macciò, Livia; Milani, Marina

doi:10.1097/dad.0b013e31828bc7ac

Cited by 16 publications

(12 citation statements)

References 21 publications

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“…When we focused our literature review on the lymphomas clearly proven to be cyclin D1 positive, we identified 16 cases of MCL with skin involvement, most of which were described as single-case reports. [10][11][12][13] Thus, despite the fact that the disease is leukemic in almost all cases (87% of cases pretreatment) and frequently shows extranodal involvement, dermotropism is not a typical feature of MCL 8,14 Most of the cases in our analysis as well as in the published literature showed systemic MCL at the time when skin manifestation occurred. However, of 16 published cases with MCL in the skin confirmed by cyclin D1 expression, 5 had involvement limited to the skin and lacked other manifestations (Table 3).…”

Section: Discussionmentioning

confidence: 76%

“…However, of 16 published cases with MCL in the skin confirmed by cyclin D1 expression, 5 had involvement limited to the skin and lacked other manifestations (Table 3). 6,[9][10][11][12][13] Similarly, we identified 1 patient with an MCL confined to the skin at initial presentation. However, subsequent dissemination of the lymphoma cannot be excluded in our patient, as the follow-up period is short.…”

Section: Discussionmentioning

confidence: 85%

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Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Wehkamp¹,

Pott²,

Unterhalt

et al. 2015

American Journal of Surgical Pathology

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Mantle cell lymphoma (MCL) is a B-cell neoplasm with a variable and generally aggressive clinical course. So far our knowledge of skin involvement of MCL is limited. To understand the clinical and histopathologic features of MCL with skin involvement, the files of the Lymph Node Registry Kiel were screened for MCL diagnosed in the skin. Over a period of 13 years, 1321 biopsy specimens were diagnosed as MCL; among them, 14 patients (1%) showed skin involvement. Of these, skin was the initial site of manifestation in 6/11 (55%) cases. One patient presented with a skin-limited lymphoma. Furthermore, 7/12 (58%) patients presented with lesions on the leg. The lymphomas were highly proliferative with blastoid cytology in 12/14 (86%) cases. Moreover, the immunophenotype with expression of BCL2 (100%), MUM-1/IRF4 (83%), and IgM (82%) and lack of CD10 (25%) and BCL6 (0%) closely resembled the features of primary cutaneous diffuse large B-cell lymphoma, leg type. Solely the expression of cyclin D1 (100%) and the presence of t(11;14) (100%) allowed a distinction from cases of primary cutaneous diffuse large B-cell lymphoma, leg type. Only 2 MCL cases with skin involvement presented with classical cytology. Interestingly, in these 2 cases skin involvement occurred simultaneously in a lesion of coexisting primary cutaneous marginal zone lymphoma. Our data suggest that clinical presentation on the leg and blastoid cytology along with high proliferation and expression of Bcl2, Mum-1/IRF4, and IgM are typical for MCL involving the skin. Lymphomas with these features might be erroneously diagnosed as diffuse large B-cell lymphoma, leg type, if cyclin D1 staining is not performed.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 85%

Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Wehkamp¹,

Pott²,

Unterhalt

et al. 2015

American Journal of Surgical Pathology

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“…It is said that cutaneous dissemination can be present in up to 17% of patients with stage IV disease . Primary cutaneous involvement by MCL has been the subject of isolated case reports . A primary cutaneous lymphoma, according to the WHO‐EORTC definition, is one that presents in the skin with no evidence of extracutaneous disease at the time of presentation.…”

Section: Discussionmentioning

confidence: 99%

“…Cutaneous involvement by MCL is very uncommon with most cases representing secondary cutaneous dissemination by systemic disease . Primary cutaneous MCL is exceedingly rare with only isolated case reports that have been published, and typically most of the patients presented with systemic lymphoma afterwards …”

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confidence: 99%

Cutaneous mantle cell lymphoma: a clinicopathologic review of 10 cases

et al. 2016

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Cutaneous mantle cell lymphoma (MCL) is exceedingly rare, almost always occurring as a dissemination of systemic MCL. To date, only 32 cases have been described. We report a series of 10 cases of MCL in the skin, and provide a comprehensive clinicopathologic review with clinical follow-up. Our cases occurred in older individuals (mean age = 70) and were more frequently in men (90%). Half of them presented in the head and neck region as a mass/nodule, and the remainder in the trunk and extremities as nodules. All patients have stage IV disease. In two of the 10 cases (20%) the cutaneous lesions preceded the diagnosis of disseminated disease. In two of the seven cases with available clinical follow-up information (33%) the skin was immediately involved after the diagnosis of MCL, and in three of the seven the skin was the first site of recurrence (mean interval = 57 months). The mean time to recurrence of the disease was 45.4 months and the overall survival was 66.3 months. Histologically six out of 10 cases (60%) had either pleomorphic or blastoid morphology (four out of 10 and two out of 10, respectively). The mean number of mitoses per 10 high-power fields was 18.44. While nine out of 10 cases expressed cyclin-D1, one case was not positive for cyclin-D1 but did label with SOX-11. Limited cytogenetic data showed trisomy 14 in one case, in addition to the t(11;14) translocation.

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“…4 Given this infrequency, and the absence of large series, we know less of about primary cutaneous mantle cell lymphoma than about other cutaneous lymphomas. There have been only 4 cases of purported primary cutaneous mantle cell lymphoma up to now.…”

Section: Discussionmentioning

confidence: 99%

“R/O Lymphoma”

LeBoit¹,

Jacobson²

2014

Pathology Case Reviews

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A 78-year-old man had a skin biopsy submitted to a dermatopathology laboratory with the clinical data of "r/o lymphoma"; as the diagnosis was unclear, it was then sent to a consultant dermatopathologist. Because of a clonal plasmacytoid population, the consultant rendered the diagnosis of marginal zone lymphoma. A subsequent telephone call from the oncologist caring for the patient revealed that there was a long history of mantle cell lymphoma, and additional studies showed that the correct diagnosis was a rare variant, mantle cell lymphoma with plasmacytic differentiation. Biopsies submitted for the diagnosis of lymphoma should include pertinent clinical information, and the failure to do so can lead to added expense and possible misdiagnosis.

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Primary Cutaneous Mantle Cell Lymphoma of the Leg With Blastoid Morphology and Aberrant Immunophenotype

Cited by 16 publications

References 21 publications

Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Cutaneous mantle cell lymphoma: a clinicopathologic review of 10 cases

“R/O Lymphoma”

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