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Cutaneous CD30 positive lymphoproliferative disorders: Diagnostic challenges
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 Cutaneous CD30 Positive Lymphoproliferative Disorders: Diagnostic Challenges

 Alexandra-Sînziana Dumitru1, Irina Mărgăritescu2, Teodora Predescu1,  Călin Giurcăneanu1,3, Ana-Maria Forsea1,3

 1. Department of Oncologic Dermatology and Allergology, Elias University Clinical Hospital, Bucharest, Romania; 2. Onco Team Diagnostic, Department of  Histopathology, Monza Hospital, Bucharest, Romania; 3. Carol Davila University of Medicine and Pharmacy, Bucharest, Romania


Introduction

Cutaneous CD30 positive lymphoproliferative disorders encompass a broad range of diseases, including primary cutaneous anaplastic large-cell lymphoma (pcALCL), lymphomatoidpapulosis, tumor transformation of mycosis fungoides or borderline disorders. These diseases have multiple clinical and histopathological variants, partly overlapping, but different prognosis and management. They must also be differentiated from the secondary skin determinations of systemic lymphomas. Thus their precise diagnosis and correct classification is essential for the patients’ best care, but is challenging for the practitioner and needs careful and close correlation between clinical and pathological evaluation. 


Case report 

G.M.C. – 70 year-old Caucasian male with 6 months history of: slowly progressing pruriginous eruption of small superficial erythematous-violaceous nodules, grouped in a circinate pattern, with central clearing and residual hyperpigmentation, in the right infra-inguinal area (Figures 1, 2).

 

Past medical history: high blood pressure, hyperthyroidism, parapsoriasis in large plaques

(5 years previously, however, without active lesions at presentation);

General clinical examination and investigations: within normal limits according to age.

 

Diagnostic workup

 Biopsy:

•diffuse and nodular lymphoid infiltrate, of large and medium CD30 positive T cells (>75% of the tumor cells) with focal epidermotropism;

      irregular, large, hyperchromatic and vesiculous nuclei (Figures 4-6)

•interspersed small reactive lymphocytes and numerous eosinophils (Figure 4 and 5)
•CD3+, CD4+, CD8-, ALK-, CD20- stainings (Figure 7).

 

Extension investigations:

•chest, abdominal and pelvis computed tomography   
•complete hematological workup                                             did not show any lymph node or other
•inguinal and axillary lymph nodes ultrasound                                      extracutaneous involvement 
 

 

Diagnosis: Primary Anaplastic Large-cell Lymphoma

 

Treatment: Radiotherapy

 

The evolution was favorable, achieving complete response and without new lesions at 4 months follow-up (Figure 3).

 

Discussion and Conclusion

Epidemiology:

pcALCL is rare, difficult to distinguish from other CD30-positive cutaneous lymphoproliferative disorders, hence the the exact incidence is not known.

In an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, 157 cases of primary, localized CD30-positive cutaneous lymphoproliferative disorder were documented over a 30-year timespan [3]:

•median age at diagnosis was 61 years (range 5 to 98 years)
•slight ♂ predominance (58%)
•most cases were diagnosed in Caucasians (87%)

Other epidemiologic reports have had similar findings [4,5].  

 

Clinical presentation:

Most patients with pcALCL present with:

•solitary/grouped nodules growing over weeks/months
•that typically ulcerate with time
•leg involvement portends a worse prognosis (5-year disease-specific survival 76% vs. 96% for other locations; surprisingly, pathologic involvement of local nodes alone does not seem to portend an adverse prognosis [6].
•multifocal disease is rare at the time of diagnosis and extracutaneous spread occurs in up to 13% of cases, at relapse [7].

 

 

Evolution and treatment:

•pcALCL lesions regress in up to 50% of cases, but rarely completely resolve [8]
•complete surgical excision with negative margins of isolated lesions is the preferable treatment; radiation is an alternative
•a choice is often made based on the location of the lesions and expertise available
•the optimal dose of radiation for pcALCL is not known; electrons are frequently used with a dose of 36 to 40 Gy and a margin of at least 2 cm, though lower doses may be equally efficacious [9].  

 

References

1.Kempf W,   A neweraforcutaneousCD30-positiveT-celllymphoproliferativedisorders,Semin.Diag.Pathol., 2017  in press
2.Kempf W, et al, EORTC, ISCL, and USCLC consensusrecommendations for thetreatment of primarycutaneous CD30-positive lymphoproliferativedisordersBlood. 2011; 13;118(15):4024-35.
3.Yu JB, Blitzblau RC, Decker RH, et al. Analysis of primary CD30+ cutaneous lymphoproliferative disease and survival from the Surveillance, Epidemiology, and End Results database. J ClinOncol 2008; 26:1483.
4.BookenN, Goerdt S, Klemke CD. Clinical spectrum of primary cutaneous CD30-positive anaplastic large cell lymphoma: an analysis of the Mannheim Cutaneous Lymphoma Registry. J Dtsch Dermatol Ges 2012; 10:331.
5.Bekkenk MW, Geelen FA, van Voorst Vader PC, 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.
6.Benner MF, Willemze R. Applicability and prognostic value of the new TNM classification system in 135 patients with primary cutaneous anaplastic large cell lymphoma. Arch Dermatol 2009; 145:1399.
7.Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol 2003; 49:1049.
8.Weaver J, Mahindra AK, Pohlman B, et al. Non-mycosis fungoides cutaneous T-cell lymphoma: reclassification according to the WHO-EORTC classification. J Cutan Pathol 2010; 37:516.
9.Yu JB, McNiff JM, Lund MW, Wilson LD. Treatment of primary cutaneous CD30+ anaplastic large-cell lymphoma with radiation therapy. Int J Radiat Oncol Biol Phys 2008; 70:1542.
10.The clinical images are from the collection of Department of Oncologic Dermatology and Allergology, Elias University Clinical Hospital, Bucharest, Romania;
11.The histopathology images are from the collection of Onco Team Diagnostic, Department of Histopathology, Monza Hospital, Bucharest, Romania.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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