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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.84 no.3 Rio de Janeiro July 2009
Dermatopathologist, Service of Pathology, Hospital Obrero No 1 Caja Nacional de Salud - La Paz, Bolivia
It is a case of fast growth tumor lesion on the posterior region of the right arm of a 37-year-old female patient. Dermatopathological and immunohistochemical characteristics are discussed to support the definite diagnosis.
Keywords: Lymphoma; Lymphoma/pathology; Skin neoplasms
This is the case of a patient with fast growth tumoral lesions located on the right arm, which presented spontaneous regression after biopsy. We discussed the histological findings and patient progression.
Female 37-year-old patient with fast growth tumoral lesion measuring 2.5 x 2 cm located on the posterior region of the right arm, which had progressed for about 3 weeks (Figure 1a). Upon removing the crust, the lesion showed rounded, red, bloody surface of elastic consistent, slightly adhered to deep planes (Figure 1b). The physical examination showed no specific findings. All support clinical tests were within the normal range.
A biopsy by 6mm punch was performed and showed dense infiltration of medium and large sized lymphoid cells, placed on medium and deep dermis levels with ulceration of corresponding epidermis and pseudo-epitheliomatous hyperplasia in one of the margins (Figure 2). By magnification, we noticed that the cells were pleomorphic, presented enlarged nuclei with irregular chromatin, prominent nucleolus, irregular nuclear membrane, scarce cytoplasm and atypical mitosis figures. Such cells were mixed with plasmocytes, neutrophils and eosinophils (Figures 3 and 4). Complementary immunohistochemical staining was positive for CD45, CD3 and CD30 in tumor cells, including paranuclear enhance (Figure 5). Staining for CD 20, CD 79a, CD 10, ALK and EMA was negative.What is your diagnosis?
Two weeks after biopsy, the patient came for a follow-up visit and there was almost complete elimination of the initial lesion (Figure 6).
Three weeks after the biopsy, a new follow-up visit showed total closure of the lesion, leaving behind only a crust (Figure 7a) and after one month, there was only atrophic scar at the lesion site (Figure 7b).
What is your diagnosis?
Primary cutaneous anaplastic large cell lymphoma.
The patient was followed up for 10 years and there was onset of some new lesions, some of them treated surgically and some with spontaneous remission (Figure 8a). In her last visit, the patient had multiple regional axillary adenopathies (Figure 8b) and significant impairment of her overall health status.
Cutaneous anaplastic large cell lymphoma, primarily cutaneous with later dissemination to regional lymphatic ganglia.
Antigen CD30 is a protein that belongs to the superfamily of tumor necrosis receptors. This antigen was initially described within Reed Sternberg cells and Hodgkin cells of Hodgkin disease and later identified within the neoplastic cells of a new group of non-Hodgkin lymphoma known as anaplastic large cell lymphomas, some of which had only cutaneous manifestations 1. The term CD30-positive large cell lymphoma was proposed to name this group of primary cutaneous large T cell lymphomas characterized by favorable prognosis. Most of the cases have cutaneous presentation. Cases described in the past as regressive atypical histiocytosis or pseudo-Hodgkin disease are part of the spectrum of cutaneous lymphoproliferative diseases that are CD30-positive 1,2.
This entity was included in the group of CD30-positive cell proliferation, which also includes lymphomatoid papulosis. It is important to emphasize that there is no defined limit between what is lymphomatoid papulosis and cutaneous anaplastic T cell lymphoma, given that there are cases of typical clinical manifestations of lymphomatoid papulosis that histologically present as anaplastic large cell lymphoma and the other way round 3,4.
Cutaneous anaplastic large cell lymphoma is normally presented in adults and in both genders. Clinically, the patients have solitary or located lesions, frequently with dark red ulcers. Spontaneous regression may be perceived in some cases. Even though the histological and cytological spectrum of these tumors shows high grade lesion, prognosis is normally favorable, except for ganglionar presentation in which the prognosis is poor 4,5.
Histologically, this neoplasm shows nodular or diffuse infiltration throughout the dermis and subcutaneous cell tissue comprised by groups of atypical large anaplastic cells with rounded nuclei, prominent nucleoli and abundant cytoplasm 6. In some cases there might be giant Reed Sternberg cells and immunoblasts and pleomorphic cells. Ulcerated lesions show epidermal hyperplasia and abundant infiltration limited by lymphocytes, plasmocytes and polymorphonuclear cells 7, 8, 9.
Complementary immunohistochemical study showed marked positive results to CD 30, CD 3 and CD 4. Sometimes, the expression of Pan T antigen may be partially or totally lost. CD15 and EMA are negative in skin lesions and sometimes EMA is present when the lesion is systemic or the skin lesions are secondary to ganglionar primary focus. ALK expression is not found in purely cutaneous cases 10.
The genetic study has showed monoclonal rearrangement of TCR genes in most cases. There is controversy about whether interchromosome translocation 2, 5 is present in skin cases 10.
Treatment may be performed using radiotherapy with or without previous surgery. Patients who have generalized lesions or ganglionar dissemination require combine chemotherapy. Prognosis after five years shows 90% survival of patients even with ganglionar involvement 9, 10.
1. Latkowski J, Heald P. Cutaneous T-cell lymphomas. In: Freedberg I, Eisen A, Wolff K, Austen K, Goldsmith L, Katz S, eds. Fitzpatricks Dermatology in General Medicine. New York: McGraw Hill; 2003. p.153757 [ Links ]
2. Grayson W, Calonje E, Mckee PH. Infectious diseases of the skin. In: McKee PH, Calonje JE, Granter SR, eds. Pathology of the skin. Philadelphia, (PA): Elsevier Mosby; 2005. p. 900 [ Links ]
3. Bartralot R, Pujol RM, Garcia-Patos V, Sitjas D, Martín- Casabona N, Coll P, et al. Cutaneous infections due to nontuberculous mycobacteria: histopathological review of 28 cases. Comparative study between lesions observed in immunosuppressed patients and normal hosts. J Cutan Pathol. 2000;27:1249 [ Links ]
4. Chandler FW, Watts JC. Pathologic diagnosis of fungal infections. Chicago (CA): American Society of Clinical Pathologists; 1987 [ Links ]
5. McKee PH, Calonje JE, Granter SR. Connective tissue tumors. In: McKee PH, Calonje JE, Granter SR, eds. Pathology of the skin. Philadephia (PA): Elsevier Mosby; 2005. p.177981 [ Links ]
6. Kuo TT, Shih LY, Tsang NM. Nasal NK/T cell lymphoma in Taiwan: a clinico-pathologic study of 22 cases, with analysis of histologic subtypes, Epstein-Barr virus LMP- 1 gene association, and treatment modalities. Int J Surg Pathol. 2004;12:37587 [ Links ]
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8. Martâºn JM, Ricart JM, Monteagudo C, Alcácer J, Pinazo I, Tomás L, et al. Primary cutaneous CD30+ anaplastic large-cell lymphomas mimicking keratoacanthomas. Clin Exp Dermatol. 2007;32:66871 [ Links ]
9. Lin JH, Lee Y. Primary cutaneous CD 30+ anaplastic large cell lymphoma with keratoacanthoma-likepseudocarcinomatous hyperplasia and marked eosinophilia and neutrophilia. J Cutan Pathol. 2004;31:45861 [ Links ]
10. Scarisbrick JJ, Calonje E, Orchard G, Child FJ, Russell- Jones R. Pseudocarcinomatous change in lymphomatoid papulosis and primary cutaneous CD 30+ lymphoma: a clinicopathologic and immunohistochemical study of 6 patients. J Am Acad Dermatol. 2001;44:23947 [ Links ]
11. Cespedes YP, Rockley PF, Flores F, Ruiz P, Kaiser MR, Elgart GW. Is there a positive relationship between CD30-positive lymphoproliferative disorders and epidermal proliferation? J Cutan Pathol. 2000;27:2715 [ Links ]
12. Courville P, Wechsler J, Thomine E, Vergier B, Fonck Y, Souteyrand P, et al. Pseudoepitheliomatous hyperplasia in cutaneous T-cell lymphoma. A clinical, histopathological and immunohistochemical study with particular interest in epithelial growth factor expression. The French Study Group on Cutaneous Lymphoma. Br J Dermatol. 1999;140:4216 [ Links ]
13. Bernier M, Bagot M, Broyer M, Farcet JP, Gaulard P, Wechsler J. Distinctive clinicopathologic features associated with regressive primary CD30 positive cutaneous lymphomas: analysis of 6 cases. J Cutan Pathol. 1997;24:15763 [ Links ]
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Conflict of interest: None
Financial funding: None
How to cite this article: Sangueza JM. Proliferación tumoral en brazo derecho. An Bras Dematol. 2009;84(3):270-4.