For citation purposes: Jaffe J, Wang Z, Salame G, Lee YC, Alagkiozidis I. Extramedullary haematopoiesis mimicking metastatic lymphadenopathy in a patient with squamous cell carcinoma of the vulva. OA Case Reports 2013 Sep 10;2(10):92.

Obstetrics and Gynaecology

 
Obstetrics & Gynecology

Extramedullary haematopoiesis mimicking metastatic lymphadenopathy in a patient with squamous cell carcinoma of the vulva

J Jaffe1, Z Wang2, G Salame1, YC Lee1, I Alagkiozidis1
 

Authors affiliations

(1) Department of Gynecologic Oncology, SUNY-Downstate Medical Center, Brooklyn, NY, USA

(2) Department of Pathology, SUNY-Downstate Medical Center, Brooklyn, NY, USA

*Corresponding author Email: alagkiozidis@gmail.com

Abstract

Introduction

We report a case of extramedullary haematopoiesis manifested as multi focal lymphadenopathy in patient with vulvar cancer. The appearance of haematopoietic elements outside the bone marrow has been associated with solid tumours.

Case report

A 60-year-old woman presented to the Emergency Department at SUNY-Downstate Hospital complaining of generalised abdominal pain. This patient was managed with radical resection of the vulvar lesion and inguinal lymph node dissection.

Conclusion

Vulvar cancer is a rare malignancy with approximately 4500 new cases and 1000 deaths annually in the United States. Thorough and systematic preoperative workup is essential before treatment planning.

Introduction

Extramedullary haematopoiesis is the appearance of haematopoietic tissue outside of the bone marrow[1]. Sites most commonly involved are the spleen, liver, lymph nodes and mediastinum[1,2]. It is usually associated with pathologic entities that are causing ineffective myelopoiesis, leading to peripheral cytopaenias such as bone marrow neoplasms or haemolytic anaemias[3,4]. Several cases of solid tumours associated with extramedullary haematopoiesis have been reported[5]. Here we report the first case of extramedullary haematopoiesis occurring in a patient with vulvar cancer.

Case report

A 60-year-old woman presented to the Emergency Department at SUNY-Downstate Hospital complaining of generalised abdominal pain. Her medical history was significant for seizure disorder. Her medications were Keppra and Gabapentin. Family history was non-contributory. Social history was significant for smoking (one pack per week). Physical examination revealed a thin woman without lymphadenopathy, clear breath sounds bilaterally and hepatomegaly (10 cm below the costal margin). Gynaecologic exam revealed a 1 ´ 1 cm area of hyperkeratosis and white discoloration on the right labia minora, 2.5 cm lateral to clitoris. Relevant laboratory findings revealed haemoglobin 12 g/dL, haematocrit 40, MCV (mean corpuscular volume) 82 fL, RBC count 5 million/mL, platelets 141,000/mL, WBC count 8490/mL, BUN (Blood Urean Nitrogen) 8 mg/dL, Cr 0.7 mg/dL, albumin 3.4 g/dL, total bilirubin 0.9 mg/dL, AST ( aspartate aminotranferase) 29 U/L, ALT (alanine aminotransferase) 16 U/L and INR (international normalized ratio) 1.2.

CT (computed tomography) abdomen/pelvis showed a 4.7 ´ 4.1 cm portocaval mass abutting the pancreatic head, hepatomegaly, portal, gastrohepatic, aortocaval lymphadenopathy up to 1.8 cm in short axis and 6.1 ´ 4.4 cm cystic lesion in the left adnexa. Chest CT showed pretracheal and subcarinal lymphadenopathy up to 2 cm in short axis. PET (positron emission tomography) scan revealed hypermetabolic areas in the neoplastic range in the left carotid sheath with SUV (standardized uptake value) of 5.8, paratracheal regions bilaterally (SUV 7.6), gastrohepatic ligament (SUV 6.8) and right vulva (SUV 5.8). The portocaval mass and the adnexal cyst were not metabolically active (Figure 1).

Whole-body positron emission tomography scan showing hypermetabolic areas in neck, mediastinum, upper abdomen and vulva.

Mediastinoscopy with biopsy of the mediastinal lymph nodes demonstrated reactive lymphadenopathy with extramedullary haematopoiesis (Figure 2). CT-guided biopsy of the portocaval mass showed haemangioma. Biopsy of the vulvar lesion showed invasive well-differentiated squamous cell carcinoma of the vulva (Figure 3). Depth of invasion on the biopsy specimen was 1 mm. Given the negative metastatic workup, we proceeded with radical local excision of the vulvar carcinoma and inguinal lymph node dissection. The surgical specimen was negative for residual tumour. All inguinal lymph nodes were negative. Final stage of the vulvar carcinoma was IA.

Biopsy of paratracheal lymph nodes. Reactive lymphadenopathy with extramedullary haematopoiesis. Cells of myeloid, erythroid and megakaryocytic lineage are identifiable (Hematoxylin and Eosin, high power).

Vulvar biopsy (right labia minora). Invasive well-differentiated squamous cell carcinoma (Haematoxylin and Eosin, high power).

Discussion

Extramedullary haematopoiesis usually occurs in the reticuloendothelial system with spleen and liver involvement being the most prominent. It has also been described in other sites such as the gastrointestinal tract, lung, pleura, skin, breast, central nervous system, adrenals, kidneys and uterus[1,5].

It is considered to be a compensatory phenomenon when associated with anaemia or space-occupying bone marrow pathologic processes[3,4]. Of note, our patient was not anaemic at presentation. Histologically, cells of myeloid, erythroid and megakaryocytic lineage are identifiable, the latter sometimes being the most obvious.

Extramedullary haematopoiesis may be associated with solid tumours. It has been identified in patients with angiomyolipoma, liposarcoma, spindle cell lipoma, cerebellar haemangioblastoma, lung carcinoma, colon cancer and renal carcinoma[3,5,6,7]. There is evidence to support that the occurrence of extramedullary haematopoiesis in patients with solid tumours without bone marrow invasion is related to growth factors that are being secreted by the tumour and stimulate hyperplasia of circulating haematopoietic precursor cells. For instance, immunohistochemistry of solid tumours has demonstrated positivity for erythropoietin[8,9,10]. This pathophysiologic mechanism is not related to the amount of the tumour since small, early-stage solid tumours have been associated with extramedullary haematopoiesis[5].

Conclusion

Here we present a case of early-stage vulvar carcinoma with concurrent extramedullary haematopoiesis in the lymph nodes. Vulvar cancer is a rare malignancy with approximately 4500 new cases and 1000 deaths annually in the United States. Thorough and systematic preoperative workup is essential before treatment planning. Extramedullary haematopoiesis should be included in the differential diagnosis in a case of vulvar cancer with diffuse lymphadenopathy, and histologic evaluation is required.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Authors contribution

All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.

Competing interests

none declared.

Conflict of interests:

none declared.

A.M.E

All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.

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