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Review
. 2023 Feb 3;18(1):12.
doi: 10.1186/s13000-023-01297-w.

Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature

Ying Yin  1 , Huaipu Liu  2 , Minghua Luo  1 , Guangyin Yu  1 , Weihua Yin #  3 , Ping Li #  4
Affiliations
Review

Primary extranodal soft tissue Lennert lymphoma (lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified): a case report and review of the literature

Ying Yin et al. Diagn Pathol. .

Abstract

Lennert lymphoma (LeL) is a rare variant of peripheral T-cell lymphoma, not otherwise specified (PTCL/NOS) that is rich in epithelioid histiocytes. LeL may pose great diagnostic and therapeutic challenges to the pathologist and clinician. Primary extranodal soft tissue LeL is even rarer and has not been reported. Herein, we report a case of LeL arising from soft tissue.A 65-year-old male presented for evaluation of a painless mass in the subcutaneous soft tissue of the left forehead. There was no invasion of the bone and no ulceration on the surface of the skin. The surrounding skin was erythematous and swollen. Grossly, the tumor was gray-red and 30 mm × 20 mm × 10 mm in size.Microscopically, the demarcation between the lesion and surrounding tissues was unclear without a capsule. The tumor invaded the surrounding striated muscle and adipose tissue. The tumor had a diffuse proliferation of small-sized atypical lymphocytes and numerous large clusters of epithelioid histiocytes. Plasma cells, eosinophils, and Hodgkin-Reed-Sternberg (HRS) cells were not identified. Rare multinucleated histiocytes were noted, and well-formed granulomas were not present. Rare mitotic figures were noted, but no necrosis. The immunophenotypic features in this case were as follows: CD2+/CD3+/CD5low+/CD7+/CD4low+/ CD8+/CD30-/CD56- in neoplastic lymphocytes; CD163+/CD31+/CK(pan)- in epithelioid histiocytes; and CD20-/CD30-/TdT-/CD5-/ALK-/S-100-/CD1α-/CD21 + 23-/SSTR2- in neoplastic lymphocytes and epithelioid histiocytes. Epstein-Barr virus (EBV)-encoded RNA in situ hybridization (EBER-ISH) was negative. The Ki-67 index was elevated to 60%. PCR showed a polyclonal pattern for IgH and a monoclonal TCR γ-chain rearrangement.The final diagnosis was PTCL/NOS, lymphoepithelioid cell variant (LeL), which arose from soft tissue and had a rare double-positive CD4low+/CD8+ immunophenotype. The patient received four cycles of cyclophosphamide, doxorubicin liposomes, vincristine, and prednisone tablets (CHOP) and was followed for 20 months. Overall treatment efficacy was achieved without lymphadenopathy, and no other discomfort or illnesses were reported.

Keywords: Lennert lymphoma; Lennert type; Peripheral T-cell lymphoma; Soft tissue.

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Conflict of interest statement

All authors declare that they have no any conflict of interests.

Figures

Fig. 1
Fig. 1
A Plain CT scan of the brain showed swelling of the subcutaneous soft tissue of the forehead with increased density and CT value of 63 HU. There was no invasion of the bone. B Grossly, the tumor was gray-red and 30 mm × 20 mm × 10 mm in size. C The cut surface of the mass was solid, grey-red, and slightly firm
Fig. 2
Fig. 2
Microscopic findings (Haematoxylin and eosin [HE] staining). A The demarcation between the lesion and surrounding tissues was unclear and lacked a capsule. B The lesion appeared multinodular in shape. Nodules were surrounded by proliferative fibrous connective tissue. C The tumor invaded the surrounding striated muscle and adipose tissue. D, E The tumor showing a diffuse proliferation of small lymphocytes and numerous large clusters of epithelioid histiocytes. F At high magnification, the proliferation of small lymphocytes displayed slight nuclear irregularities. Plasma cells, eosinophils, and Hodgkin-Reed-Sternberg (HRS) cells were not identified. Rare multinucleated histiocytes were noted, and well-formed granulomas were not present. Rare mitotic figures and no necrosis were observed. (HE staining, A 7.3×; B 20×; C 100×; D 200×; E 200×; F 400×)
Fig. 3
Fig. 3
Immunohistochemical findings and EBER in situ hybridization. Immunohistochemical stains in small lymphocytes for CD2, CD3, CD5 (a few), CD7, CD4 (a few), CD8, and granzyme B are positive. Over-expressed for Ki-67. Immunohistochemical stains in epithelioid cells for CD163 and CD31 are diffusely positive, and negative for CK(pan). In situ hybridization for EBER was negative. (A CD2, 400×; B CD3, 400×; C CD5, 400×; D CD7, 400×; E CD4, 400×; F CD8, 400×; G granzyme B, 400×; H Ki-67, 200×; I CD163, 400×; J CD31, 400×; K CK(pan), 50×; L EBER-ISH, 100×)
Fig. 4
Fig. 4
Immunohistochemical findings for other negative antibodies. Immunohistochemical stains for CD20, CD30, TdT, CD56, ALK, S-100, CD1α, CD21 + 23, and SSTR2 are negative. (A CD20, 200×; B CD30, 400×; C TdT, 50×; D CD56, 50×; E ALK, 50×; F S-100, 400×; G CD1α, 50×; H CD21 + 23, 200×; I SSTR2, 400×)
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