Emphysematous endometritis in stage III endometrial cancer

Abstract

We report a case of emphysematous endometritis in a 65-year-old patient who has stage III, high-grade, poorly differentiated endometrial cancer; she was on chemotherapy. The patient developed pyogenic emphysematous endometritis complicated by hypovolemic shock and sepsis. She was admitted to the intensive care unit for treatment with vasopressors and antibiotics. The shock was successfully managed and her hospital course was otherwise unremarkable.

Background

Endometrial pneumatosis, also known as emphysematous or pneumopolycystic endometritis, is an extremely rare condition. To our knowledge, it is reported only four times previously in the literature.^1–4 Pneumatosis of the female genital tract is generally rare; it most frequently develops in the vagina or cervix in a condition that is known as emphysematous vaginitis or cervicitis, respectively.^5–7 Ovarian pneumatosis has also been reported in one case.⁸ Emphysematous inflammations of the abdomen and pelvis are fatal conditions that require aggressive management. Endometrial pneumatosis is an ambiguous condition of uncertain aetiology. Unless complicated, it appears to be self-limited with no known pathological complications, so it is considered a rare but benign condition.^{2 3}

Case presentation

A 65-year-old African American woman with a history of stage III non-resectable, high-grade, poorly differentiated glassy cell endometrial cancer on chemotherapy (carboplatin and paclitaxel) was admitted to the hospital with generalised abdominal pain, bloating and anorexia of 1-week duration. The patient also had complaints of nausea and vomiting in addition to vaginal discharges. She denied fever, chills or change in her bowel habits.

On presentation, her blood pressure was 58/45 mm Hg, heart rate was 134 beats per minute, respiratory rate was 22 breaths per minute, temperature was 36.2°C and oxygen saturation was 99% on room air. Physical examination revealed a distended abdomen, generalised abdominal tenderness with no localising signs and normal bowel sounds.

Investigations

Laboratory findings showed lactic acid of 11.3 mmol/L (normal: 0.4–2.0 mmol/L), haemoglobin of 80 g/L (baseline: 80–90 g/L) (normal: 140–170 g/L), creatinine of 3.08 mg/dL (baseline: 0.6–0.7 mg/dL) (normal: 0.6–1.1 mg/dL), blood urea nitrogen of 33 mg/dL (normal: 7–25 mg/dL), aspartate transaminase of 50 IU/L (normal: 7–55 IU/L), alkaline phosphatase of 154 IU/L (normal: 44–147 IU/L) and albumin of 2.1 g/dL (normal: 3.4–5.4 g/dL). Platelets were 194 x 10⁹/L (normal: 150 - 400 x 10⁹/L). Mean corpuscular volume was 96.8 fL (normal: 80–100 fL), mean corpuscular haemoglobin was 28.9 pg (normal: 27.5–33.2 pg), mean corpuscular haemoglobin concentration was 29.9 g/dL (normal: 31–35 g/dL) and red cell distribution width was 18.4% (11.8%–14.5%). White blood cell (WBC) count was 5.1 x 10⁹/L (4.5-11 x 10⁹/L)and absolute neutrophil count was 3.0 x 10⁹/L (normal: 1.6 - 7.0 x 10⁹/L). Bands were 0.4 x 10⁹/L (normal: 0.0-0.5 x 10⁹/L). Blood cultures showed no growth, and urinalysis was negative for an infection.

ECG showed sinus tachycardia. Chest X-ray showed no evidence of acute cardiopulmonary processes. CT scan of the abdomen and pelvis with contrast showed interval development of ground opacities in the right lower lobe, which were not present in previous CT scan; small perihepatic ascites, which was also new since the prior scan; and the presence of air within the endometrial cavity, with a small left locule of air abutting the uterus which was concerning for secondary infection of the previously seen endometrial cancer or emphysematous endometritis. CT scan findings are shown in figure 1.

Figure 1.

Different views of the CT scan of abdomen and pelvis with contrast showing markedly distended endometrial cavity with fluid with the presence of air within the endometrial cavity. A small left locule of air abutting the uterus, which indicates emphysematous endometritis and transmural infectious process which is likely reaching the uterine serosa. It also shows metastatic lymphadenopathies in the left periaortic region and bilateral pelvis.

Urinary analysis with the microscopic examination showed a WBC count of 2–5 cells/high-power field and 1+ bacteriuria, and urine cultures confirmed negative urinary tract infection (UTI). The patient does not have intrauterine instrumentation as confirmed by the history, pelvic examination and CT scan findings.

The cancer was previously staged III and deemed unresectable because the CT scan of the abdomen/pelvis showed extensive pelvic lymphadenopathy mainly in the left periaortic and bilateral iliac lymph nodes, which were suggestive of metastasis (figure 1); the same findings were found in previous CT scans. The case was discussed at the multidisciplinary tumour conference with gynaecological oncology’s recommendations that the tumour was non-resectable.

Differential diagnosis

The differential diagnosis included septic shock due to other sources of infection like pneumonia; the patient denied clinical symptoms of pneumonia. Other sources of infection were ruled out by detailed history, physical examination, the above findings on the CT scan and negative sepsis workup.

UTI was ruled out by negative urine analysis and urine culture. No instrumentation history and no foreign bodies were identified in the CT scan.

Treatment

The patient was given 2 L of intravenous fluids. Due to concern about infectious endometritis, she was started on vancomycin and cefepime while in the emergency department, blood cultures were sent, a central line was placed and the patient was started on norepinephrine. The patient was admitted to the intensive care unit for further care and management. After 3 days, the patient was gradually weaned off norepinephrine and she was transferred to the telemetry medical floor in a stable condition.

Outcome and follow-up

Due to ischaemic acute tubular necrosis, creatinine continued to worsen and then plateaued. Because of anaemia, the patient was given two units of packed red blood cells when haemoglobin dropped to less than 7.0 g/dL. Other laboratory findings continued to improve during the hospital stay.

Haematology/oncology, infectious diseases, nephrology and palliative medicine consultants were involved. Intravenous antibiotics were continued, and the patient was discharged on oral levofloxacin, doxycycline and metronidazole for a total duration of 28 days. The treatment options were discussed with the patient who decided to follow up with her medical and gynaecological oncologists as outpatient.

Discussion

The first case of pneumopolycystic endometritis was reported in 1960 by Perkins. It was a case of a 32-year-old Mexican woman who had a cystic lesion after uncomplicated labour of her second child; that lesion involved the entire cervix, the endocervix, the vagina, the vulva and the endometrium. The patient had numerous cysts throughout the vulva, vagina and cervix. Gross evaluation of the uterus showed similar endometrial cysts. Pelvic X-rays showed innumerable gas-containing cystic structures bordering the entire uterus. Vaginal and cervical histopathology evaluation revealed emphysematous vaginitis/cervicitis with multiple empty and variably sized cystic spaces present within the edematous stroma which were lined by endothelial cells, multinucleated giant cells or connective tissue stroma, in addition to mild nonspecific inflammatory infiltrate. Histopathological evaluation of the endometrium was not performed. The patient’s condition spontaneously resolved and her 2-week postpartum follow-up was unremarkable.¹

Another case was reported by Val-Bernal et al for a previously healthy 49-year-old para 2 woman who was non-pregnant. She presented with menometrorrhagia of 1-year duration. Ultrasonography revealed a diffusely thickened endometrium of 24 mm, an anechoic cystic structure of 14.9×13.7 mm in diameter in the posterior uterine wall and a 28 mm cyst of liquid content in the left ovary. The patient underwent dilatation and curettage. The histopathological examination showed proliferative endometrium, endometrial hyperplasia and empty cystic stromal spaces throughout the endometrial stroma. The authors concluded that their report was the first to describe histopathology of pneumatosis limited to the endometrium as Perkins’s histopathological studies were only on the cervix, not the endometrium.¹ The patient’s hospital course was uncomplicated with unremarkable 3-month follow-up. Although this case was reported under the title of ‘pneumopolycystic endometritis’ similar to the case described by Perkins, the authors concluded that ‘endometrial pneumatosis’ instead of ‘endometritis’ may have been a more applicable term as there was no significant inflammatory component.²

The third reported case by Chua et al was of a 43-year-old healthy pregnant woman at 16 weeks’ gestational age who had a 1-year history of worsening menorrhagia and dyspareunia. Imaging showed an enlarged uterus with a large solid mass in the anterior uterine body consistent with an intramural fibroid. The endometrium had a normal thickness of 9 mm. Later, the patient underwent a total abdominal hysterectomy and bilateral salpingectomy without complications. Gross evaluation of the specimen revealed a multicystic cobblestone appearance of the entire anterior and posterior endometrial surfaces. Histological examination showed numerous irregular and partially collapsed vesicular spaces within the superficial endometrial stroma extending close to the surface epithelium. Residual intact endometrial cysts in the gross specimen were punctured under water, and some appeared to release gas bubbles consistent with entrapped air, which raised the suspicion for pneumatosis-like process. Clinically, the condition was self-limited and postoperative follow-up was unremarkable. Since the patient had not had any prior instrumentation or endometrial biopsies, the authors concluded that the large leiomyoma may have led to mucosal disruption or produced obstruction of the uterine cavity, causing high intraluminal pressure because the tumour did not involve the endometrium directly and the pneumatosis involved the anterior and posterior mucosal surfaces equally.³

In contrast to the case reported by Val-Bernal et al² where a single hypoechoic space was seen on ultrasonography, the case reported by Chua et al³ demonstrated numerous 2–3 mm spaces within the endometrial mucosa that were beyond the imaging resolution.

Another case of a 40-year-old woman with a history of abnormal uterine bleeding was reported by Podoll et al. Microscopic examination revealed numerous cyst-like spaces throughout the endometrial stroma in a proliferative endometrium. The endometrial stromal cells created an appearance of small oval nuclei. The spaces were variable in size, from microcysts to large ectatic spaces. No evidence of inflammation or cellular debris was prominent. Unlike the case reported by Val-Bernal et al,² no radiological findings were available to correlate with histology and determine whether there was any endometrial lesion prior to the biopsy. The authors concluded the possibility that the stromal spaces represent tissue procurement or processing artefact could not be entirely excluded.⁴

In the four previously reported cases, endometrial pneumatosis appears to be self-limited with no pathological sequelae; this is comparable with our patient’s hospital course after the complication of shock had been successfully treated.

Patient’s perspective.

I have been fighting endometrial cancer for about 6 months. The fact that I have an aggressive cancer sometimes scares me. The experience of chemotherapy treatment including its side effects and complications carries its own hurdles. When I see my daughters and grandchildren, I always feel that I must continue the close follow-up and treatment. When the doctors tell me that the current complication is rare, I feel nervous, but I remember that I promised myself to face any complication during this battle.

Learning points.

• To shed light on a rare condition ‘endometrial pneumatosis’ by including a literature review of similar cases.

• To clarify an unclear relationship between the diagnosis of endometrial cancer and an unusual complication ‘endometrial pneumatosis’.

• Endometrial pneumatosis is usually self-limited unless complicated by genitourinary tract or systemic infections which can be life-threatening, especially in an immunocompromised host.