Monocytic Sarcoma of the Sclera

Abstract

A patient with acute monocytic leukaemia and gross ocular involvement successfully treated with local irradiation is described. Leukaemic infiltration of the sclera developed at a time when the patient appeared to be in haematological remission. Despite the frequency of extramedullary disease in monocytic leukaemia the diagnosis of monocytic sarcoma may be very difficult.

Is acute myeloid leukemia a liquid tumor?

“…20 The French-American-British subtypes of AML most commonly associated with cutaneous (in up to 50% of cases) and noncutaneous EML are acute myelomonocytic (AML-M4) and acute monocytic (AML-M5) leukemia. 9,[21][22][23][24][25][26] In APL, EML is more likely to occur at relapse, with incidence estimated at approximately 3%. EML is rare at APL diagnosis, with half of cases affecting the skin.…”

Irradiation for Conjunctival Granulocytic Sarcoma

“…This case demonstrates that this entity may also occur in older patients as an initial symptom of acute myelogenous leukemia. Not many cases of orbital myeloic sarcoma in elderly patients have been reported [12,18,30]. Furthermore, our patient developed extramedullary leukemic tumor formation in the conjunctiva as a rare site of manifestation within the orbita.…”

“…Also during further hematological relapses no recurrence within the irradiated orbital fields occurred. Only few cases of ocular granulocytic sarcoma treated with local irradiation have been described, but all of them reported good results [18,29,30]. Watkins et al [30] and van Veen et al [29] each described a case of complete remission of a large solid extraconal orbital granulocytic sarcoma in adult patients after local irradiation with 36 and 40 Gy, respectively, together with chemotherapy.…”

“…Watkins et al [30] and van Veen et al [29] each described a case of complete remission of a large solid extraconal orbital granulocytic sarcoma in adult patients after local irradiation with 36 and 40 Gy, respectively, together with chemotherapy. Murray et al [18] reported a case of an elderly patient with diffuse scleral monocytic sarcoma who was treated successfully with a total dose of 20 Gy in nine fractions without concurrent chemotherapy. Two other case reports regarding local irradiation of lymphoblastic sarcoma of the iris and conjunctiva, respectively, described excellent results with a total dose of 20 Gy [5,21].…”

Ocular Granulocytic Sarcoma: A Case Report and Literature Review of Ocular Extramedullary Acute Myeloid Leukemia

“…Among the 5 previously mentioned patients receiving external beam radiation, all had somewhat favorable ocular outcomes (although not necessarily favorable overall systemic outcomes). 2,4,5,7,10 …”