|LETTER TO THE EDITOR
|Year : 2017 | Volume
| Issue : 6 | Page : 1076-1077
Isolated myeloid sarcoma of cervix: Aleukemic presentation
Meenakshi Kamboj, Anila Sharma, Gurudutt Gupta, Sunil Pasricha
Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
|Date of Web Publication||13-Dec-2017|
Dr. Meenakshi Kamboj
Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5, Rohini, New Delhi - 110 085
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kamboj M, Sharma A, Gupta G, Pasricha S. Isolated myeloid sarcoma of cervix: Aleukemic presentation. J Can Res Ther 2017;13:1076-7
A 43-year-old female presented with bleeding per vaginum of 1-month duration. There were no other symptoms and all blood investigations were unremarkable.
Computed tomography and magnetic resonance imaging showed a large mass lesion in uterine cervix measuring 9.5 cm × 7.5 cm × 5.7 cm involving both the lips, upper two-third of vagina, bilateral parametria, and left pelvic wall. A separate nodular lesion in labial soft tissue and enlarged bilateral inguinal nodes were seen.
Cervical biopsy showed intact mucosa, with submucosa replaced by a diffusely infiltrative poorly differentiated tumor, destroying the endocervical glands [Figure 1]. The cells showed convoluted nuclei, indiscernible cytoplasm, and frequent apoptosis, with a sprinkling of eosinophils and neutrophils. Morphologic differentials included neuroendocrine carcinoma, lymphoma, and a poorly differentiated carcinoma. Immunohistochemistry (IHC) performed showed the expression of leukocyte common antigen (CD45), CD68, and myeloperoxidase (MPO), and negative cytokeratin, synaptophysin, chromogranin, CD3, CD20, and CD10 [Figure 2]. MIB-1 labeling index (Ki-67) was 60%.
|Figure 1: Myeloid sarcoma of the cervix. (a) Section showing endocervical mucosa with submucosa infiltrated by a poorly differentiated tumor in diffuse sheets (H and E, ×40); (b) sheets of tumor cells with starry sky pattern and destruction of endocervical glands (H and E, ×200); (c) tumor cells with convoluted nuclei and indiscernible cytoplasm with a sprinkling of eosinophils and neutrophils (H and E, ×400)|
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|Figure 2: Immunohistochemistry of myeloid sarcoma. Tumor cells express myeloperoxidase (a) and CD45 (b) and negative for cytokeratin (c). Cytokeratin highlights the residual cervical mucosa|
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Bone marrow aspirate and biopsy performed were unremarkable. A final diagnosis of primary myeloid sarcoma (MS) was thus established. Treatment plan with neoadjuvant chemotherapy and radiotherapy, followed by surgery and subsequent chemotherapy was offered. However, the patient was lost to further follow-up.
Previously termed chloroma and granulocytic sarcoma, MS is the currently recommended term by the World Health Organization. MS is an extramedullary tumor of malignant granulocytic or monocytic progenitor cells that may occur concurrently, or precede acute or chronic myeloid leukemia, or myelodysplastic and myeloproliferative disorders., It is seen in 1.4–9% of patients with acute myeloid leukemia (AML). MS may indicate blastic transformation of chronic myeloproliferative disorder or a relapse of AML, and can manifest de novo less commonly.
MS can virtually involve any anatomic site, but is commonly seen in the bone, periosteum, soft tissue, lymph nodes, and skin. Female genital tract forms a rare site, with ovary involved most commonly, followed by cervix and uterus.
Histologically, the diagnosis of MS can be challenging when presentation is unusual, or neoplastic cells are immature and occur in the absence of AML. Morphologic differentials include inflammatory lesions, lymphomas, undifferentiated or metastatic carcinoma, epithelioid sarcoma, and in ovary granulosa cell and germ cell tumor. Eosinophilic precursors form an extremely helpful feature when present, which however can be absent in 50% of cases.
IHC can successfully identify the majority of cases with CD68 as the most commonly expressed marker followed by MPO, CD117, CD99, lysozyme, CD34, TdT, CD56, CD61, CD30, glycophorin A, and CD4. Genetic association has been seen with a variety of chromosomal aberrations, in particular, t(8;21), inv(16), and +8.
The optimal treatment is unclear due to its rarity and is treated similar to leukemia with induction chemotherapy. In a study of 24 patients of MS, 5-year survival rate was 20%. Isolated MS shows frequent extramedullary recurrence and almost always proceed to frank leukemia, thus requiring long-term follow-up.
In conclusion, the primary diagnosis of MS presenting as isolated cervical mass is challenging, and requires an extended IHC panel.
The authors thank Dr. Jatin S. Gandhi for his kind support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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