SCImago Journal & Country Rank
This journal is a member of, and subscribes to the principles of, the Committee on Publication Ethics (COPE)
2021, Volume 37, Number 3, Page(s) 249-253
[ Abstract ] [ PDF ] [ Similar Articles ] [ E-Mail to Author ] [ E-Mail to Editor ]
DOI: 10.5146/tjpath.2020.01517
Primary Myeloid Sarcoma of Ovary: A Case Report and Review of the Literature
Sevda AKYOL1, Fatma OZ ATALAY2
1Department of Pathology, Hakkari Yuksekova State Hospital, HAKKARI, TURKEY
2Department of Bursa Uludag University Medicine Faculty, BURSA, TURKEY
Keywords: Myeloid sarcoma, Ovary, Female genital neoplasms
Myeloid sarcoma (granulocytic sarcoma or chloroma) is a tumor formed by myeloid precursor cells in any localization other than the bone marrow. It can occur without underlying acute myeloid leukemia (AML) or other myeloid neoplasms. Herein, we present a forty-two-year-old female patient who underwent surgery because of a left adnexal mass. Microscopic examination of the specimen revealed cord-like arrangement of the tumor cells with a diffuse growth of small blue cells effacing the ovarian stroma. Adult granulosa cell tumor was in the differential given the scanty cytoplasm of the tumor and in fact was the diagnosis of the referring institution. Further microscopic evaluation with immunohistochemical analysis at our institution revised the diagnosis to myeloid sarcoma. Myeloid sarcoma is a difficult tumor to diagnose due to its rarity, especially in the absence of a history of leukemia, and correct tissue diagnosis is essential for its treatment.
Myeloid sarcoma (granulocytic sarcoma, chloroma) is a tumor formed by myeloid precursor cells in any localization other than the bone marrow 1. It was first described in the literature by Burns in 1811 2. When the tumor is exposed to oxygen, it turns green. Due to this color of the tumor, it was named chloroma derived from the Greek word chlorosis (green) 3.

Myeloid sarcoma can occur without underlying acute myeloid leukemia (AML) or other myeloid neoplasms. It can be seen anywhere on the body but skin, lymph node, gastrointestinal tract, bone, and testicle placement is common 4,5. Only a few cases of myeloid sarcoma localized in the female genital system (cervix, vagina, vulva, ovary, and endometrium) have been reported in the literature 6-8. In this case report, we presented a case of primary ovarian myeloid sarcoma without an apparent, previously diagnosed hematologic malignancy.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Case Presentation
    A forty-two-year-old female patient was admitted to another center with pain in the abdomen. The patient was followed up with psoriasis and had no other known systemic diseases. Upon detecting a mass in the left adnexal area in the radiological images, total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed.

    Grossly, the tumor was located in the left adnexa and was 9 cm in the greatest dimension. The cut surface was solid with areas of necrosis. The microscopic examination showed a diffuse infiltrative growth pattern with tumor cells focally forming cord-like structures. The tumor was poorly differentiated with 50 mitoses per 10 high power fields (HPF). The widespread necrosis seen by gross examination was also appreciated by microscopic evaluation. The tumor cells were mostly uniform with little cytoplasm and hyperchromatic nuclei, and focally cells with eosinophilic cytoplasm with vesicular nuclei and conspicuous nucleoli and ill-defined cytoplasmic borders were present (Figure 1A-D).

    Click Here to Zoom
    Figure 1: A) Areas with sheet-like infiltration by small-blue tumor cells (H&E; x40). B-C) Tumor cells consisting of cord structures separated by fine fibrous septa (H&E; x100&200). D) The presence of eosinophilic myelocytes among tumor cells is remarkable (H&E; x200).

    The tumor was initially thought to be a granulosa cell tumor and referred to our institution for further workup. Given the poorly-differentiated nature of the tumor, the differential was broad and included lymphoma, metastatic breast carcinoma, poorly differentiated carcinoma (metastatic or primary), melanoma, or an unusual presentation of a malignant mesenchymal tumor. Tumor cells showed widespread positive reactivity with LCA, CD117, MPO, CD43, lysozyme, CD34, and CD68 in the immunohistochemical stainings (Figure 2A-D). No immune reactivity was observed with PanCK, vimentin, CD3, CD20, EMA, SF-1, WT-1, inhibin, or calretinin. As a result of the morphological and immunohistochemical studies, the case was reported as myeloid sarcoma.

    Click Here to Zoom
    Figure 2: Strong positivity to immunohistochemical staining with A) CD34 (IHC; x100). B) CD117 (IHC; x100). C) Lysozyme (IHC; x100). D) MPO (IHC; x100).

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Discussion
    Ovarian tumors are classified histopathologically as epithelial tumors, mesenchymal tumors, mixed epithelial and mesenchymal tumors, sex cord-stromal tumors, mixed sex cord-stromal tumors, germ cell tumors, germ cellsex cord-stromal tumors, mesothelial tumors, soft tissue tumors, lymphoid and myeloid tumors, miscellaneous tumors, tumor-like lesions and secondary tumors 9. Ninety percent of malignant ovarian tumors are of epithelial origin 10. However, when the literature was examined, it was observed that there were less than 50 cases of myeloid neoplasms of the ovary.

    The first primary ovarian myeloid sarcoma was published in 1959 11. Twenty-five cases have been reported as case reports 12-33. Two relevant series comprising 19 cases were published 34-35. The patient ages in the case reports ranged from 3 months to 58 years. The mean age of the patients was 32.1 years 18-33. Most of the patients were in the reproductive age. The age of our patient was 42 years. The patients presented with complaints of abdominal pain (8/15), mass (2/15), cough (1/15), and vaginal bleeding (1/15), and the pathology was detected in 3 patients (3/15) during a routine check 20-33. The tumor was located in the left ovary in 5 cases, the right ovary in 8 cases, and bilaterally in 3 cases 15,19,22-33. In our case, it was located in the left ovary. The tumor diameter ranged from microscopic size to 25 cm 25,28. The size of the tumor in our case was 9x8.5 cm.

    Ovarian myeloid sarcoma may occur before AML, simultaneously with AML, as a result of the recurrence of AML, or as an isolated tumor mass. It may also be associated with myelodysplastic syndrome or myeloproliferative neoplasm34. Three of the cases were found to be isolated myeloid sarcoma 15,25,33. After myeloid sarcoma diagnosis, AML developed in 8 cases 16,21-23,26,30-32. Bone marrow involvement was seen simultaneously with myeloid sarcoma in 2 cases 20,28. Before the mass developed in the ovary, six patients had AML and 1 patient had chronic myeloid leukemia 14,17,19,24,29. The time of the systemic disease with medullary involvement following the initial myeloid sarcoma diagnosis ranged from just after surgery to 18 months afterwards 26,31.

    Diagnosing myeloid sarcoma in the absence of a history of leukemia can be challenging. It is reported that only 44% of myeloid sarcoma cases can be diagnosed correctly at the first examination 36. In our case, it was misdiagnosed as a granulosa cell tumor at another center at the first examination. Granulosa cell tumor may be considered first when a blue cell tumor with scant cytoplasm is seen in the ovary. However, the cells are angulated nuclei and some have grooves similar to coffee beans in granulosa cell tumor. The tumor may exhibit various architectural patterns such as trabecular, diffuse and microfollicular. Even if a highgrade transformation is encountered in some tumors, there are still low-grade areas containing typical morphological features separated by an abrupt transition. Abundant eosinophilic cytoplasm and prominent nuclear atypia are observed in tumor cells in the high-grade areas. The nuclei can have a bizarre multinucleated form 37.

    Myeloid sarcomas are less differentiated and the differential diagnosis spectrum is therefore quite wide. Diffuse large B cell lymphoma, Burkitt’s lymphoma, undifferentiated carcinoma, metastatic lobular carcinoma, small cell carcinoma of the hypercalcemic type, granulosa cell tumor, and dysgerminoma can be included in the differential diagnosis. Immunohistochemical studies can be very helpful during the differential diagnosis. LCA positive staining and CD3 and CD20 negative staining eliminate the diagnosis of lymphoma. Negative staining with panCK excludes the diagnosis of metastatic carcinoma and undifferentiated carcinoma. Other diagnoses are excluded with CD56, synaptophysin, calretinin, inhibin, SF-1, and PLAP negativity. CD34, MPO, CD117, CD68, and lysozyme positivity also support the diagnosis of myeloid sarcoma.

    In conclusion, we presented a case of myeloid sarcoma in the ovary, which has been published in the literature only in very small numbers. It is important to know the patient’s history and consider this tumor entity to make a correct diagnosis.

    The authors declare no conflict of interest.

    Both authors contributed to titles such as concept, design, data collection, analysis, literature review, writing, approval.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • References

    1) Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Beau MML, Orazi A, Siebert R. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4. ed; IARC Press: 2017. 167-8.

    2) Burns A. Observations of Surgical Anatomy in Head and Neck. Edinburgh: Thomas Royce, 1811.

    3) King A. Case of chloroma. Monthly Journal of Medical Science. 1853;8:97-104.

    4) Falini B, Lenze D, Hasserjian R, Coupland S, Jaehne D, Soupir C, Liso A, Martelli MP, Bolli N, Bacci F, Pettirossi V, Santucci A, Martelli MF, Pileri S, Stein H. Cytoplasmic mutated nucleophosmin (NPM) defines the molecular status of a significant fraction of myeloid sarcomas. Leukemia. 2007;21:1566-70.

    5) Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, Piccaluga PP, Agostinelli C, Asioli S, Novero D, Bisceglia M, Ponzoni M, Gentile A, Rinaldi P, Franco V, Vincelli D, Pileri A Jr, Gasbarra R, Falini B, Zinzani PL, Baccarani M. Myeloid sarcoma: Clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21:340-50.

    6) Garcia MG, Deavers MT, Knoblock RJ, Chen W, Tsimberidou AM, Manning JT Jr, Medeiros LJ. Myeloid sarcoma involving the gynecologic tract: A report of 11 cases and review of the literature. American Journal of Clinical Pathology 2006;125:783-90.

    7) Guile MW, Ross MV, Sewell C. Granulocytic sarcoma of the endometrium. Female Patient. 2008;33:45-6.

    8) Ahmed N, Al-Badawi I, Chebbo W, Chaudhri N, El-Gohary G. Case report: Myeloid sarcoma of the vulva post-bone marrow transplant presenting as isolated extra medullary relapse in a patient with acute myeloid leukemia. Hematology/Oncology and Stem Cell Therapy. 2012;5:118-21.

    9) Kurman RJ, Carcangiu ML, Herrington CS, Young RH. WHO Classification of Tumours of Female Reproductive Organs. 4th ed. Lyon: IARC, 2014.

    10) Torre LA, Trabert B, DeSantis CE, Miller KD, Samimi G, Runowicz CD, Gaudet MM, Jemal A, Siegel Rl. Ovarian cancer statistics, 2018. Ca Cancer J Clin. 2018;68:284-96.

    11) Hinkamp JF, Szanto PB. Chloroma of the ovary. Am J Obstet Gynecol. 1959;78:812-6.

    12) Schäfer RJ, Hill K, Reitz H. Myelosarkom (leukotisches Sarkom) des Ovars vor Manifestation einer Leukose [Myelosarcoma (leucotik sarcoma) of the ovary preceeding Leukemia (Author’s Transl)]. Zentralbl Allg Pathol 1974;118:467-72.

    13) Castilone HC. Chloroma of ovaries: Report of a case. Bull Millard Fillmore Hosp Buffalo. 1962;9:98-102.

    14) Sagar TG, Maitreyan V, Majhi U, Shanta V. Ovarian granulocytic sarcoma in chronic granulocytic leukaemia with myeloblastic crisis. J Assoc Physicians India. 1989;37:542-3.

    15) Stefenelli T, Linkesch W, Radaszkiewicz T. Myelosarkom des Ovars [Myelosarcoma of the ovary]. Wien Klin Wochenschr. 1991;103:717-8.

    16) Kobayashi M, Ohno R, Yamada K, Hirabayashi N. A case of granulocytic sarcoma of the ovary with minimal involvement of the bone marrow, liver and spleen. Rinsho Ketsueki. 1977;18:1154-9.

    17) Adnène Laatiri M, Zahra K, Bedoui M, Mellouli S, Ktata M, Remadi S, Kortas M, Khlif A. Tumeur ovarienne et diagnostic de leucémie myeloide chronique. A propos d’un cas [Ovary tumor in chronic myeloid leukaemia. Case report]. Gynecol Obstet Fertil. 2007;35:434-6.

    18) Ballon SC, Donaldson RC, Berman ML, Swanson GA, Byron RL. Myeloblastoma (granulocytic sarcoma) of the ovary. Arch Pathol Lab Med. 1978;102:474-6.

    19) Szomor A, Passweg JR, Tichelli A, Hoffmann T, Speck B, Gratwohl A. Myeloid leukemia and myelodysplastic syndrome relapsing as granulocytic sarcoma (chloroma) after allogeneic bone marrow transplantation. Ann Hematol. 1997;75:239-41.

    20) Magliocco AM, Demetrick DJ, Jones AR, Kossakowska AE. Granulocytic sarcoma of the ovary. An unusual case presentation. Arch Pathol Lab Med. 1991;115:830-4.

    21) Tripathi R, Sharma B, Chaturvedi KU, Khurana N, Mala YM. Granulocytic sarcoma of the female genital tract: Report of a case with an unusual presentation. Gynecol Obstet Invest. 2005;59:189-91.

    22) Morgan ER, Labotka RJ, Gonzalez-Crussi F, Wiederhold M, Sherman JO. Ovarian granulocytic sarcoma as the primary manifestation of acute infantile myelomonocytic leukemia. Cancer. 1981;48:1819-24.

    23) Pressler H, Horny HP, Wolf A, Kaiserling E. Isolated granulocytic sarcoma of the ovary: Histologic, electron microscopic, and immunohistochemical findings. Int J Gynecol Pathol. 1992;11:68-74.

    24) Yamamoto K, Akiyama H, Maruyama T, Sakamaki H, Onozawa Y, Kawaguchi K. Granulocytic sarcoma of the ovary in patients with acute myelogenous leukemia. Am J Hematol. 1991;38:223-5.

    25) Roy P, Biswas B, Sankari S, Manipadam MT, Jose R. Primary granulocytic sarcoma of ovary. J Obstet Gynaecol India. 2012;62:91-3.

    26) Aguiar RC, Pozzi DH, Chamone DA. Granulocytic sarcoma of the ovary in a nonleukemic patient. Haematologica. 1993;78:53-5.

    27) Ding J, Li H, Qi YK, Wu J , Liu Z, Huang B, Chen W. Ovarian granulocytic sarcoma as the primary manifestation of acute myelogenous leukemia. Int J Clin Exp Pathol. 2015;8:13552-6.

    28) Kahn RM, Gordhandas S, Chapman-Davis E, Margolskee E, Matrai C, Chadburn A, Ritchie E. Acute myeloid leukemia presenting as myeloid sarcoma with a predisposition to the gynecologic tract. Case Rep Oncol Med. 2019;2019:4189275.

    29) George SM, Chandran N, Malik AK. Granulocytic sarcoma of ovary in a post allogenic bone marrow transplant patient - A diagnostic challenge to the anatomic pathologist. Case report and review of literature. J Obstet Gynaecol. 2016;36:567-70.

    30) Mehta J, Kane S. Primary granulocytic sarcoma of ovary-a rare presentation. Leuk Lymphoma. 2004;45:845-7.

    31) Choi JY, Kim HY, Kang MG, Shin JK, Lee WS, Song HN. Bilateral ovarian granulocytic sarcoma as the primary manifestation of acute myelogenous leukemia treated with allogenic stem cell transplantation: A case report. Medicine (Baltimore) 2019;98:e18390.

    32) Wang X, Liu H, Wu Z, Xu X, Chen X, Zhai Z, Sun Z. A case of acute promyelocytic leukemia presenting with a nonleukemic granulocytic sarcoma of the ovary, with subsequent development of acute myeloid leukemia associated with t(8;21). Leuk Res. 2009;33:580-2.

    33) Sreejith G, Gangadharan VP, Elizabath KA, Preetha S, Chithrathara K. Primary granulocytic sarcoma of the ovary. Am J Clin Oncol. 2000;23:239-40.

    34) Zhang X, Huang P, Chen Z, Bi X, Wang Y, Wu J. Vulvar myeloid sarcoma as the presenting symptom of acute myeloid leukemia: A case report and literature review of Chinese patients, 1999-2018. Diagn Pathol. 2019;14:126.

    35) Oliva E, Ferry JA, Young RH, Prat J, Srigley JR, Scully RE. Granulocytic sarcoma of the female genital tract: A clinicopathologic study of 11 cases. Am J Surg Pathol. 1997;21:1156-65.

    36) Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell RE, Bennett JM. Granulocytic sarcoma: A clinicopathologic study of 61 biopsied cases. Cancer. 1981;48:1426-37.

    37) Fashedemi Y, Coutts M, Wise O, Bonhomme B, Baker G, Kelly PJ, Soubeyran I, Catherwood MA, Croce S, McCluggage WG. Adult granulosa cell tumor with high-grade transformation. Am J Surg Pathol. 2019;43:1229-38.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References

  • Copyright © 2021 The Author(s). This is an open-access article published by the Federation of Turkish Pathology Societies under the terms of the Creative Commons Attribution License that permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited. No use, distribution, or reproduction is permitted that does not comply with these terms.

    [ Top ] [ Abstract ] [ PDF ] [ Similar Articles ] [ E-Mail to Author ] [ E-Mail to Editor ]