Granulocytic Sarcoma

Diagnosis

Granulocytic Sarcoma

Diagnosis

Granulocytic Sarcoma

History

6-year-old girl with 2-month evolution of left mandible mass.Lytic lesion on plain X-Rays, likely ameloblastoma

Findings

Expansile lytic lesion in the left mandibule, centered at the level of teeth 33-34, measuring 4.3 cm AP x 3.3 cm LL x 6.3 cm CC. Extension in the masticator and retromaxillozygomatic spaces. No invasion of the pterygoid plates or maxillary sinus.
Heterogeneous uptake, in part due to recent biopsy.
SUVmax up to 5.1.
One or two left sided I-B lymph nodes are demonstrated, inactive. Remaining whole body FDG PET/CT within normal limits.

Discussion

Biopsy of the lesion in this particular case revealed the presence of tumoral infiltration with a low Ki-67, with positivity for CD45, CD34, C68, negativity of CD3, CD4, CD20, CD30, AE1-AE3, MYO- D1, SALL4, PHOX-2B and normal INI1. Diagnosis of granulocytic sarcoma was made. Bone marrow biopsy was normal.
The differential diagnosis of lesions involving the mandible is extensive. In children the most common acquired ones are cysts, infections , ameloblastoma, fibrous dysplasia and Langherans cell Histiocytosis. Sarcomas are infrequent. Mandibular lesions are classified as benign or malignant, odontogenic and nonodontogenic. Furthermore, they are subdivided into solid or cystic tumors. Finally, the location of the lesion in the mandibule helps identification.
Granulocytic sarcoma (GS) also called chloroma or myeloid sarcoma is a tumor composed by mature or immature blast cells and represents extramedullary location of leukemia. It is more frequently associated with acute myeloid leukemia ( AML ) than acute lymphoid leukemia (ALL). It can antedate or be in association with the leukemia. In some occasions, it will be the presentation of relapse.
Isolated GS is extremely rare , less than 2 cases per million in adults and 0.7 in children. More than 85% of patients will progress to acute leukemia without systemic treatment in addition to local treatment.
Every tissue can be affected by GS. The most common locations are the skin, the head and neck region, in particular the orbital region, the anterior mediastinum, the gastrointestinal tract and the bone. Localizations are the same for either de novo isolated GS or GS associated with concomitant AML.
FDG PET/CT have shown to be effective to identify GS in patients with known AML. Only in a minority of patients uptake is low, limiting their evaluation. Only a few patients have been imaged following treatment or for the evaluation of relapse and in all cases, FDG PET/CT proved to be useful.
In isolated GS it allows identification of additional lesions not suspected clinically and indicate pinpoint the best site for biopsy.
Isolated bone GS is uncommon as only one other case, involving the temporal bone, imaged with FDG PET/CT, has been reported .
This particular case was challenging as GS mimicked more frequent mandible tumor.

Key points

Differential Diagnosis (adults and childrens):
Odontogenic Lesions
Cysts
Cementoblastoma
Ameloblastoma
Odontogenic Myxoma
Keratocystic odontogenic tumor
Nonodontogenic Lesions:
Simple bone cyst
Aneurysmal bone cyst
Fibrous Dysplasia
Infection
Langerhans Cell Histiocytosis
Cherubism
Vascular lesions and malformations
Metastatic disease
Primary lymphoma of the bone
Chloroma
Sarcomas
Giant cell granulomaPlasmocytoma

References

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