|Year : 2022 | Volume
| Issue : 1 | Page : 54-55
Anaplastic large-cell lymphoma presenting as large ulcerated tumors and small papules in a 15-year-old girl
Takamitsu Matsuzawa1, Juri Shu2, Yuumi Nakamura3, Moeko Hino4, Jun-Ichiro Ikeda5, Makoto Sugaya6, Hiroyuki Matsue7
1 Department of Dermatology, Chiba University Graduate School of Medicine, Chiba, Japan
2 Department of Dermatology, Chiba University Graduate School of Medicine, Chiba; Department of Dermatology, Kimitsu Chuo Hospital, Kisarazu, Japan
3 Department of Dermatology, Chiba University Graduate School of Medicine, Chiba; Cutaneous Immunology, Immunology Frontier Research Center, Osaka University, Osaka, Japan
4 Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan
5 Department of Diagnostic Pathology, Chiba University Graduate School of Medicine, Chiba, Japan
6 Department of Dermatology, International University of Health and Welfare, Chiba, Japan
7 Department of Dermatology, Chiba University Graduate School of Medicine; Medical Mycology Research Center, Chiba University, Chiba, Japan
|Date of Submission||24-Oct-2021|
|Date of Decision||06-Jan-2022|
|Date of Acceptance||07-Jan-2022|
|Date of Web Publication||30-Mar-2022|
Dr. Takamitsu Matsuzawa
Department of Dermatology, Chiba University Graduate School of Medicine, 1-8-1, Inohana, Chuo-Ku, Chiba
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Matsuzawa T, Shu J, Nakamura Y, Hino M, Ikeda JI, Sugaya M, Matsue H. Anaplastic large-cell lymphoma presenting as large ulcerated tumors and small papules in a 15-year-old girl. Dermatol Sin 2022;40:54-5
|How to cite this URL:|
Matsuzawa T, Shu J, Nakamura Y, Hino M, Ikeda JI, Sugaya M, Matsue H. Anaplastic large-cell lymphoma presenting as large ulcerated tumors and small papules in a 15-year-old girl. Dermatol Sin [serial online] 2022 [cited 2023 Mar 21];40:54-5. Available from: https://www.dermsinica.org/text.asp?2022/40/1/54/341355
Primary cutaneous CD30+ T-cell lymphoproliferative disorders include primary cutaneous anaplastic large-cell lymphoma (PCALCL) and lymphomatoid papulosis (LyP). Children with PCALCL appear to have clinicopathological features similar to those of adults. PCALCL usually manifests as a solitary tumor or grouped nodules with a tendency to ulcerate. Spontaneous regression of cutaneous lesions was occasionally observed in patients with PCALCL. On the other hand, LyP usually presents as recurrent grouped or disseminated papules and small nodules on the trunk and/or limbs, which spontaneously regress within a few weeks, sometimes leaving postinflammatory hyperpigmented macules and varioliform scars.,, We report a case of PCALCL in a 15-year-old girl with skin lesions of various sizes, ranging from a small papule to a large ulcerated tumor. The same clone was detected by T-cell receptor (TCR) γ-chain gene rearrangement using DNA from a papular lesion and a large tumor.
A 15-year-old girl presented to our clinic with a large ulcerated tumor (6 cm in diameter) on the right buttock [Figure 1]. Physical examination also revealed multiple red-brown papules and postinflammatory pigmentation on the trunk and limbs [Figure 1]. These papules started 2 years before and spontaneously regressed and recurred. However, one papule on her right buttock gradually increased in size over the past 3 months, forming a large tumor. Laboratory examination showed no atypical lymphocytes in the peripheral blood and no antibodies against human T-cell leukemia virus type 1. Her serum lactate dehydrogenase level was normal. Histopathological examination of the tumor revealed an ulcerated skin lesion with massive infiltration of large atypical and pleomorphic cells with abundant mitoses throughout the dermis [Figure 1]. The epidermotropism of lymphocytes was mild in the remaining epidermis. Immunohistochemical staining showed that most of the large cells were positive for CD30, CD8, and granzyme B but negative for CD3 and CD4 [Figure 1]. Large cells did not express CD20 or anaplastic lymphoma kinase (ALK) [Figure 1]. The proliferation index Ki-67 was positive in approximately 80% of cells [Figure 1]. Monoclonal TCRγ gene rearrangement was detected in the tumor sample using a multiplex polymerase chain reaction (LSI Medience, Tokyo, Japan) [Figure 2]. Although positron emission tomography/computed tomography (PET/CT) showed high 18F-fluorodeoxyglucose uptake in the tumor, there was no evidence of extracutaneous lesions. Therefore, we diagnosed the patient with PCALCL, and the entire tumor was surgically excised. Interestingly, the same clonality pattern by TCRγ gene rearrangement was detected in the papule on the left upper abdomen as the tumor on the right buttock [Figure 2], suggesting that papules were also PCALCL. She developed another large tumor on the left axilla and a nodule on the right forearm 3 months and 2 years, respectively, after excision of the tumor on the right buttock [Figure 1]. Histological findings of these skin lesions were similar to those of the first tumor on the buttock.
|Figure 1: (a-c) Clinical pictures. (a) A reddish, ulcerated tumor (6 cm in diameter) and a red-brown papule adjacent to the tumor on the right buttock. (b and c) Multiple papules and postinflammatory pigmentation on (b) the left upper abdomen and (c) the thighs. (d) Hematoxylin–eosin staining of the tumor on the buttock. Massive infiltration of large atypical and pleomorphic cells with abundant mitoses was observed throughout the entire dermis. (e-h) Immunohistochemical staining for (e) CD30, (f) CD8, (g) anaplastic lymphoma kinase, and (h) Ki-67 of the large cells. (i) An ulcerated tumor on the left axilla. (Original magnification: [d-h] ×200).|
Click here to view
|Figure 2: Multiplex polymerase chain reaction for the T-cell receptor γ region (Vγlf, Vγ10/Jγ) in DNAs from the tumor on the right buttock (upper graph) and the papule on the left upper abdomen (lower graph). The vertical and horizontal axis indicates relative fluorescence units and product size (base pairs, bp), respectively. The sizes of the polymerase chain reaction-amplified products were the same (black arrow: 166 bp and 205 bp).|
Click here to view
PCALCL shows dense infiltrates of large pleomorphic, anaplastic, or immunoblast-like cells, of which, by definition, more than 75% express CD30. Tumor cells in PCALCL do not usually express ALK. CD8+ PCALCL is less common than CD4+ PCALCL. Epidermotropic infiltrates of atypical CD8+ CD30+ lymphocytes can be seen in LyP type D. However, in our case, mild epidermotropism and massive infiltration of atypical cells throughout the dermis were observed. Clonal rearrangement of TCR genes is detected in most of the PCALCL cases. The clinicopathological and genetic findings in our case corresponded to those of PCALCL. Furthermore, the involvement of extracutaneous organs, such as lymph nodes, was not detected by PET/CT. Therefore, we diagnosed the tumor on the buttock as PCALCL.
The clinical course of approximately 2 years of recurrent, self-healing papules suggested the diagnosis of LyP. Patients with LyP have the risk of a second neoplasm such as mycosis fungoides, PCALCL, and/or Hodgkin lymphoma. Although our case may be PCALCL following LyP, a clonal relationship between the tumor and the papule and relatively few papules or nodules present at one time led us to conclude that the small papules were cutaneous manifestations of PCALCL. Indeed, the patient currently presents with one or two papules or nodules at each visit, which is rare in LyP.
It has been reported that a high Ki-67 index is inversely related to clinical prognosis. Our patient developed a large tumor that required total resection in the hospital. Although it is difficult to decide whether the papules should be called LyP or a small skin lesion of PCALCL, we may safely say that our patient should be carefully followed up considering the risk of a second neoplasm or the progression of PCALCL.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kempf W, Kerl K, Mitteldorf C. Cutaneous CD30-positive T-cell lymphoproliferative disorders-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg 2018;37:24-9.
Ceppi F, Pope E, Ngan B, Abla O. Primary cutaneous lymphomas in children and adolescents. Pediatr Blood Cancer 2016;63:1886-94.
Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al.
Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: A report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 2000;95:3653-61.
Kempf W. CD30+lymphoproliferative disorders: Histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol 2006;33 Suppl 1:58-70.
Martinez-Cabriales SA, Walsh S, Sade S, Shear NH. Lymphomatoid papulosis: An update and review. J Eur Acad Dermatol Venereol 2020;34:59-73.
van Dongen JJ, Langerak AW, Brüggemann M, Evans PA, Hummel M, Lavender FL, et al.
Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: Report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia 2003;17:2257-317.
Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Paediatric cutaneous lymphomas: A review and comparison with adult counterparts. J Eur Acad Dermatol Venereol 2015;29:1696-709.
Nasit JG, Patel SC. Primary Cutaneous CD8(+) CD30(+) anaplastic large cell lymphoma: An unusual case with a high Ki-67 index – A short review. Indian J Dermatol 2015;60:373-7.
] [Full text]
[Figure 1], [Figure 2]