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Testicular enlargement as a presenting feature of monocytic leukemia in an infant
T e s t i c u l a r E n l a r g e m e n t as a P r e s e n t i n g F e a t u r e of M o n o c y t i c L e u k e m i a in an I n f a n t By J.P. Roberts and J.D. A t w e l l
Southampton, England 9 A c u t e monocytic leukemia is u n c o m m o n and unusual in its propensity to present w i t h e x t r a m e d u l l a r y involvement. W e describe testicular e n l a r g e m e n t in an infant as the presenting f e a t u r e of this disease.
9 1989 by W.B. Saunders Company. I N D E X W O R D S : M o n o c y t i c leukemia; testicular enlargement.
N F I L T R A T I O N of the testis with leukemic cells is a common finding when there is generalized diseaseJ Eight percent of leukemic relapses following medical treatment present with testicular enlargement. 2 The primary presentation of monocytic leukemia with testicular enlargement in an infant has not previously been reported, although a similar presentation has been described in a 2-year-old child. 3
I
CASE REPORT
An 8-month-old boy presented with bilaterally enlarging testicles noted in the preceding 2 months. He had been well otherwise, although the parents had noticed some irregularity of the scalp. There was no significant family or past medical history. On examination, the testes were firm and uniformly enlarged, measuring 5 x 3 cm (Fig 1A and B). In addition, he had coarsened facial features with mild gum and palatal hypertrophy. Several nodules were present over the vertex on palpation of the scalp. There was no skin involvement, lymphadenopathy, or hepatosplenomegaly. His peripheral white blood cell count was normal at 9.8 x 10/L. (6.4% monocytes) with no blast cells. A skull x-ray was normal, but a radionuclide bone scan showed diffusely increased uptake in the mandible, maxilla, and skull with normal activity in the remainder of the skeleton (Fig 2). The
Fig 2.
Technetium radionuclida bone scan of skull lesions.
diagnosis of acute monocytic leukemia was confirmed by biopsy of his testes and lesions from the skull. Aspiration of his bone marrow showed only a minor degree of infiltration with monocytic cells. He was treated with chemotherapy (acute myeloid leukemia protocol l0 regimen) and by irradiation of the testes. He developed pneumocystis pneumonia but has since responded well to his chemotherapy. When in remission he is due to receive an autologous bone marrow transplant. DISCUSSION
From the Wessex Paediatric Surgical Centre, Southampton General Hospital, Southampton, England. Address reprint requests to J.P. Roberts, Wessex Paediatric Surgical Centre, Tremona Rd, Southampton, England. 9 1989 by W.B. Saunders Company. 0022-3468/89/2412-0020503.00/0
Acute monocytic leukemia is uncommon, comprising 1% to 8% of nonlymphocytic leukemias in adults and children. 4 In children the incidence is highest in those under 10 years of age, the majority occurring in the first year of life. 5
Fig 1. (A) Preoperative and (B) intraoperative appearances of the testes.
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Journal of Pediatric Surgery, Vo124, No 12 (December), 1989: pp 1306-1307
MONOCYTIC LEUKEMIA IN AN INFANT
1307
A characteristic feature is the frequent involvement of extramedullary sites such as the gingiva, skin, and lymph nodes. 3'6s Although presentation with extramedullary masses and little peripheral blood or bone marrow involvement m a y occur, most patients have features of bone marrow failure on presentationfl A review of the literature shows one similar case of monocytic leukemia presenting as testicular enlarge-
m e n t in a 2-year-old boy. 3 This patient also had hepatosplenomegaly and disseminated intravascular coagulation at presentation, and he survived only a few weeks. Although our patient has had a good response to chemotherapy so far, the prognosis in these patients remains p o o r ]
REFERENCES
1. Reid H, Marsden HB: Gonadal infiltration in children with leukaemia and lymphoma.J Clin Pathol 33:722-729, 1980 2. Stoffel TJ, Nesbit ME, Levitt SH: Extramedullary involvement of the testes in childhood leukemia, Cancer 35:1203-1211, 1975 3. Peterson L, Dehner LP, Brunning RD: Extramedullary masses as presenting features of acute monoblastic leukemia. Am J Clin Pathol 75:140-148, 1981 4. Shaw MT: The distinctive features of acute monocytic leukemia. Am J Hematol 4:97-103, 1978 5. Tobelem MD, Jacquillat C, Chastang C, et al: Acute mono-
blastic leukemia: Clinical and biologic study of 74 cases. Blood 55:71-76, 1980 6. Bain B, Manoharan A, Lampert I, et aI: Lymphoma-like presentation of acute monocytic leukaemia. J Clin Pathol 36:559565, 1983 7. Darbyshire PJ, Smith HF, Oakhill A, et al: Monocytic leukemia in infancy: A review of eight children. Cancer 56:1584-1589, 1985 8. Shaw MT: Monocytic leukaemias. Hum Pathol t 1:215-227, 1980
Southampton, England 9 A c u t e monocytic leukemia is u n c o m m o n and unusual in its propensity to present w i t h e x t r a m e d u l l a r y involvement. W e describe testicular e n l a r g e m e n t in an infant as the presenting f e a t u r e of this disease.
9 1989 by W.B. Saunders Company. I N D E X W O R D S : M o n o c y t i c leukemia; testicular enlargement.
N F I L T R A T I O N of the testis with leukemic cells is a common finding when there is generalized diseaseJ Eight percent of leukemic relapses following medical treatment present with testicular enlargement. 2 The primary presentation of monocytic leukemia with testicular enlargement in an infant has not previously been reported, although a similar presentation has been described in a 2-year-old child. 3
I
CASE REPORT
An 8-month-old boy presented with bilaterally enlarging testicles noted in the preceding 2 months. He had been well otherwise, although the parents had noticed some irregularity of the scalp. There was no significant family or past medical history. On examination, the testes were firm and uniformly enlarged, measuring 5 x 3 cm (Fig 1A and B). In addition, he had coarsened facial features with mild gum and palatal hypertrophy. Several nodules were present over the vertex on palpation of the scalp. There was no skin involvement, lymphadenopathy, or hepatosplenomegaly. His peripheral white blood cell count was normal at 9.8 x 10/L. (6.4% monocytes) with no blast cells. A skull x-ray was normal, but a radionuclide bone scan showed diffusely increased uptake in the mandible, maxilla, and skull with normal activity in the remainder of the skeleton (Fig 2). The
Fig 2.
Technetium radionuclida bone scan of skull lesions.
diagnosis of acute monocytic leukemia was confirmed by biopsy of his testes and lesions from the skull. Aspiration of his bone marrow showed only a minor degree of infiltration with monocytic cells. He was treated with chemotherapy (acute myeloid leukemia protocol l0 regimen) and by irradiation of the testes. He developed pneumocystis pneumonia but has since responded well to his chemotherapy. When in remission he is due to receive an autologous bone marrow transplant. DISCUSSION
From the Wessex Paediatric Surgical Centre, Southampton General Hospital, Southampton, England. Address reprint requests to J.P. Roberts, Wessex Paediatric Surgical Centre, Tremona Rd, Southampton, England. 9 1989 by W.B. Saunders Company. 0022-3468/89/2412-0020503.00/0
Acute monocytic leukemia is uncommon, comprising 1% to 8% of nonlymphocytic leukemias in adults and children. 4 In children the incidence is highest in those under 10 years of age, the majority occurring in the first year of life. 5
Fig 1. (A) Preoperative and (B) intraoperative appearances of the testes.
1306
Journal of Pediatric Surgery, Vo124, No 12 (December), 1989: pp 1306-1307
MONOCYTIC LEUKEMIA IN AN INFANT
1307
A characteristic feature is the frequent involvement of extramedullary sites such as the gingiva, skin, and lymph nodes. 3'6s Although presentation with extramedullary masses and little peripheral blood or bone marrow involvement m a y occur, most patients have features of bone marrow failure on presentationfl A review of the literature shows one similar case of monocytic leukemia presenting as testicular enlarge-
m e n t in a 2-year-old boy. 3 This patient also had hepatosplenomegaly and disseminated intravascular coagulation at presentation, and he survived only a few weeks. Although our patient has had a good response to chemotherapy so far, the prognosis in these patients remains p o o r ]
REFERENCES
1. Reid H, Marsden HB: Gonadal infiltration in children with leukaemia and lymphoma.J Clin Pathol 33:722-729, 1980 2. Stoffel TJ, Nesbit ME, Levitt SH: Extramedullary involvement of the testes in childhood leukemia, Cancer 35:1203-1211, 1975 3. Peterson L, Dehner LP, Brunning RD: Extramedullary masses as presenting features of acute monoblastic leukemia. Am J Clin Pathol 75:140-148, 1981 4. Shaw MT: The distinctive features of acute monocytic leukemia. Am J Hematol 4:97-103, 1978 5. Tobelem MD, Jacquillat C, Chastang C, et al: Acute mono-
blastic leukemia: Clinical and biologic study of 74 cases. Blood 55:71-76, 1980 6. Bain B, Manoharan A, Lampert I, et aI: Lymphoma-like presentation of acute monocytic leukaemia. J Clin Pathol 36:559565, 1983 7. Darbyshire PJ, Smith HF, Oakhill A, et al: Monocytic leukemia in infancy: A review of eight children. Cancer 56:1584-1589, 1985 8. Shaw MT: Monocytic leukaemias. Hum Pathol t 1:215-227, 1980