BRIEF REPORT Pediatric Dermatology Vol. 32 No. 4 e156–e158, 2015
Tumor Stage Mycosis Fungoides in a Child Abstract: Mycosis fungoides is uncommon in children and most often presents as stage IA/IB. We present a case of stage IIB mycosis fungoides in a 13year-old boy and discuss diagnostic examination and treatment considerations.
Mycosis fungoides (MF) is rare in children but most often presents during the first decade (1). The clinical spectrum of pediatric MF is variable and includes patch, plaque, hypopigmented, and unilesional presentations; the last two occur more frequently in the pediatric population than in the adult population. The adult population tends to present with more classic patches, plaques, and tumors of traditional MF (2). Nearly all pediatric MF diagnoses are early stage (IA/IB); only two stage IIB cases have been reported (3). A 13-year-old boy presented with a 6-month history of a mildly pruritic rash consisting of erythematous patches on the trunk and extremities covering 5% to 10% of his total body surface area and a 1-cm tumor on the left thigh (Fig. 1). The boy had had prior treatment with intermittent topical steroids for presumed pityriasis rosea. An incision and drainage had been attempted on the thigh lesion, which had been thought to be a cyst. Doxycycline was prescribed with minimal improvement. The patient’s medical and family histories were otherwise unremarkable. Histologic examination of the tumor revealed an atypical epidermotropic lymphoid infiltrate extending into the dermis (Fig. 2), composed predominantly of a CD3+ T-cell population with a high CD4:CD8 ratio. CD2 and CD5 were preserved, with a mild loss of CD7. A significant percentage of lesional cells were CD30+. Polymerase chain reaction for T-cell receptor gamma chain rearrangement confirmed the presence of a clonal population. A biopsy specimen from a patch site demonstrated an
DOI: 10.1111/pde.12570
e156
infiltrate with the same T-cell receptor gene rearrangement clone. Computed tomography of the neck, chest, abdomen, and pelvis and positron emission tomography did not show evidence of malignancy. Bone marrow biopsy did not reveal an abnormal lymphoid population. Clinical and histopathologic findings were consistent with stage IIB cutaneous T-cell lymphoma. Lymphomatoid papulosis was also considered in the differential diagnosis, but MF was favored because there was never a spontaneous remission of any of the lesions, and later tumor biopsies demonstrated a loss of CD30 positivity with treatment and subsequent recurrence. Biopsies did not demonstrate large-cell transformation, a morphologic increase in the size of cerebriform cells to large variants, a characteristic that portends a poor prognosis in patients with tumor-stage MF (4). There are no standard treatment recommendations for childhood-onset MF, although several treatment options have been used with reported success. Topical steroids and light therapy (ultraviolet B [UVB], narrowband UVB, or psoralen plus UVA [PUVA]) are commonly used in the treatment of pediatric MF (1). Topical nitrogen mustard and excision and local radiotherapy of unilesional MF have also been described (2). Complete remission of stage IIB disease after localized electron beam radiation to tumors then whole-body PUVA combined with interferon alfa has been reported (3). Allogenic stem cell transplantation (SCT) for peripheral T-cell non-Hodgkin lymphoma in children has been reported, with acceptable overall survival (5), although its effectiveness and safety in this population remain unclear. The prognosis with tumor-stage MF in children is poorly characterized. At the time of presentation this patient had a single tumor and within several months had developed a second small tumor. Skin-directed therapies (SDTs), including narrowband UVB and nitrogen mustard, and systemic therapies including allogenic SCT were considered and discussed with the patient’s parents. Meanwhile, he started SDT with topical corticosteroids and narrowband UVB and had a near-complete response. Taking into consideration his significant improvement, unknown prognosis, and numerous risks of allogenic SCT, a conservative approach was continued while observing the patient closely. Advanced-stage pediatric MF is exceedingly rare. Our case underscores the need for a worldwide database providing information on the natural course of MF in children, allowing one to make informed
© 2015 Wiley Periodicals, Inc.
Brief Report e157
A
B
D C
Figure 1. Tumor-stage mycosis fungoides. (A, C) A tumor on the patient’s right thigh was biopsied. (B) Low- and (D) highpower hematoxylin and eosin (H&E) stains are shown. H&E-stained sections showed an atypical lymphoid infiltrate exhibiting epidermotropism and a dense underlying nodular architecture. Lesional cells were large and pleomorphic.
Figure 2. Tumor-stage mycosis fungoides. Immunohistochemical stains of the biopsied tumor show that CD4+ cells far outnumber CD8 cells. CD30 highlights approximately 30% of lesional cells. Ki67 highlights approximately 80% of lesional cells.
decisions about therapy, including the risk of subsequent second primary malignancy. Additionally, even in children, the degree of suspicion should be high when treating dermatoses recalcitrant to standard therapy. CONFLICT OF INTEREST LMG serves on advisory boards for Celgene and Seattle Genetics.
REFERENCES 1. Pope E, Weitzman S, Ngan B et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010;14:1–6. 2. Ben-Amitai D, Michael D, Feinmesser M et al. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Derm Venereol 2003;83:451–456. 3. Hodak E, Amitay-Laish I, Feinmesser M et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with
e158 Pediatric Dermatology Vol. 32 No. 4 July/August 2015
frequent follicular involvement. J Am Acad Dermatol 2014;70:993–1001. 4. Diamandidou E, Colome-Grimmer M, Fayad L et al. Transformation of mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis. Blood 1998;92: 1150–1159. 5. Giulino-Roth L, Ricafort R, Kernan NA et al. Ten-year follow-up of pediatric patients with non-Hodgkin lymphoma treated with allogeneic or autologous stem cell transplantation. Pediatr Blood Cancer 2013;60:2018– 2024. Brittany O’Neill Dulmage, B.S., B.A.* Jennifer Villase~ nor-Park, M.D., Ph.D.† Jonhan Ho, M.D.† Larisa J. Geskin, M.D.‡ Lisa M. Grandinetti, M.D.†,†
*School of Medicine and †Department of Dermatology and Dermatopathology Unit, University of Pittsburgh, Pittsburgh, Pennsylvania, ‡Department of Dermatology, School of Medicine, Columbia University, New York, New York Address correspondence to Larisa J. Geskin, M.D., Department of Dermatology, Columbia University Medical Center, 161 Fort Washington Avenue, 12th Floor, New York, NY 10032, or e-mail: [email protected]. †
Lisa M.Grandinetti passed away since the writing of this article.
Tumor Stage Mycosis Fungoides in a Child Abstract: Mycosis fungoides is uncommon in children and most often presents as stage IA/IB. We present a case of stage IIB mycosis fungoides in a 13year-old boy and discuss diagnostic examination and treatment considerations.
Mycosis fungoides (MF) is rare in children but most often presents during the first decade (1). The clinical spectrum of pediatric MF is variable and includes patch, plaque, hypopigmented, and unilesional presentations; the last two occur more frequently in the pediatric population than in the adult population. The adult population tends to present with more classic patches, plaques, and tumors of traditional MF (2). Nearly all pediatric MF diagnoses are early stage (IA/IB); only two stage IIB cases have been reported (3). A 13-year-old boy presented with a 6-month history of a mildly pruritic rash consisting of erythematous patches on the trunk and extremities covering 5% to 10% of his total body surface area and a 1-cm tumor on the left thigh (Fig. 1). The boy had had prior treatment with intermittent topical steroids for presumed pityriasis rosea. An incision and drainage had been attempted on the thigh lesion, which had been thought to be a cyst. Doxycycline was prescribed with minimal improvement. The patient’s medical and family histories were otherwise unremarkable. Histologic examination of the tumor revealed an atypical epidermotropic lymphoid infiltrate extending into the dermis (Fig. 2), composed predominantly of a CD3+ T-cell population with a high CD4:CD8 ratio. CD2 and CD5 were preserved, with a mild loss of CD7. A significant percentage of lesional cells were CD30+. Polymerase chain reaction for T-cell receptor gamma chain rearrangement confirmed the presence of a clonal population. A biopsy specimen from a patch site demonstrated an
DOI: 10.1111/pde.12570
e156
infiltrate with the same T-cell receptor gene rearrangement clone. Computed tomography of the neck, chest, abdomen, and pelvis and positron emission tomography did not show evidence of malignancy. Bone marrow biopsy did not reveal an abnormal lymphoid population. Clinical and histopathologic findings were consistent with stage IIB cutaneous T-cell lymphoma. Lymphomatoid papulosis was also considered in the differential diagnosis, but MF was favored because there was never a spontaneous remission of any of the lesions, and later tumor biopsies demonstrated a loss of CD30 positivity with treatment and subsequent recurrence. Biopsies did not demonstrate large-cell transformation, a morphologic increase in the size of cerebriform cells to large variants, a characteristic that portends a poor prognosis in patients with tumor-stage MF (4). There are no standard treatment recommendations for childhood-onset MF, although several treatment options have been used with reported success. Topical steroids and light therapy (ultraviolet B [UVB], narrowband UVB, or psoralen plus UVA [PUVA]) are commonly used in the treatment of pediatric MF (1). Topical nitrogen mustard and excision and local radiotherapy of unilesional MF have also been described (2). Complete remission of stage IIB disease after localized electron beam radiation to tumors then whole-body PUVA combined with interferon alfa has been reported (3). Allogenic stem cell transplantation (SCT) for peripheral T-cell non-Hodgkin lymphoma in children has been reported, with acceptable overall survival (5), although its effectiveness and safety in this population remain unclear. The prognosis with tumor-stage MF in children is poorly characterized. At the time of presentation this patient had a single tumor and within several months had developed a second small tumor. Skin-directed therapies (SDTs), including narrowband UVB and nitrogen mustard, and systemic therapies including allogenic SCT were considered and discussed with the patient’s parents. Meanwhile, he started SDT with topical corticosteroids and narrowband UVB and had a near-complete response. Taking into consideration his significant improvement, unknown prognosis, and numerous risks of allogenic SCT, a conservative approach was continued while observing the patient closely. Advanced-stage pediatric MF is exceedingly rare. Our case underscores the need for a worldwide database providing information on the natural course of MF in children, allowing one to make informed
© 2015 Wiley Periodicals, Inc.
Brief Report e157
A
B
D C
Figure 1. Tumor-stage mycosis fungoides. (A, C) A tumor on the patient’s right thigh was biopsied. (B) Low- and (D) highpower hematoxylin and eosin (H&E) stains are shown. H&E-stained sections showed an atypical lymphoid infiltrate exhibiting epidermotropism and a dense underlying nodular architecture. Lesional cells were large and pleomorphic.
Figure 2. Tumor-stage mycosis fungoides. Immunohistochemical stains of the biopsied tumor show that CD4+ cells far outnumber CD8 cells. CD30 highlights approximately 30% of lesional cells. Ki67 highlights approximately 80% of lesional cells.
decisions about therapy, including the risk of subsequent second primary malignancy. Additionally, even in children, the degree of suspicion should be high when treating dermatoses recalcitrant to standard therapy. CONFLICT OF INTEREST LMG serves on advisory boards for Celgene and Seattle Genetics.
REFERENCES 1. Pope E, Weitzman S, Ngan B et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010;14:1–6. 2. Ben-Amitai D, Michael D, Feinmesser M et al. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Derm Venereol 2003;83:451–456. 3. Hodak E, Amitay-Laish I, Feinmesser M et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with
e158 Pediatric Dermatology Vol. 32 No. 4 July/August 2015
frequent follicular involvement. J Am Acad Dermatol 2014;70:993–1001. 4. Diamandidou E, Colome-Grimmer M, Fayad L et al. Transformation of mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis. Blood 1998;92: 1150–1159. 5. Giulino-Roth L, Ricafort R, Kernan NA et al. Ten-year follow-up of pediatric patients with non-Hodgkin lymphoma treated with allogeneic or autologous stem cell transplantation. Pediatr Blood Cancer 2013;60:2018– 2024. Brittany O’Neill Dulmage, B.S., B.A.* Jennifer Villase~ nor-Park, M.D., Ph.D.† Jonhan Ho, M.D.† Larisa J. Geskin, M.D.‡ Lisa M. Grandinetti, M.D.†,†
*School of Medicine and †Department of Dermatology and Dermatopathology Unit, University of Pittsburgh, Pittsburgh, Pennsylvania, ‡Department of Dermatology, School of Medicine, Columbia University, New York, New York Address correspondence to Larisa J. Geskin, M.D., Department of Dermatology, Columbia University Medical Center, 161 Fort Washington Avenue, 12th Floor, New York, NY 10032, or e-mail: [email protected]. †
Lisa M.Grandinetti passed away since the writing of this article.
