Ashindoitiang Et Al 2024 Large Subserous Uterine Leiomyoma Presenting as Intraabdominal Tumor a Case Report
Ashindoitiang Et Al 2024 Large Subserous Uterine Leiomyoma Presenting as Intraabdominal Tumor a Case Report
Ashindoitiang Et Al 2024 Large Subserous Uterine Leiomyoma Presenting as Intraabdominal Tumor a Case Report
Rare Tumors
Volume 16: 1–8
Large subserous uterine leiomyoma © The Author(s) 2024
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presenting as intraabdominal tumor: A case sagepub.com/journals-permissions
DOI: 10.1177/20363613241285089
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report
Abstract
Uterine leiomyomas are common benign gynecological tumors due to the overgrowth of uterine smooth muscle. Pe-
dunculated uterine leiomyoma occurs when the mass is in continuity with the uterus with a stalk and may grow either
within the uterine cavity or outside of the uterus and may mimic ovarian neoplasms or intraabdominal tumors. Presented is
a 28-year-old woman with a progressive abdominal swelling in the past 9 months seen at the surgical outpatient of our
facility. Preoperative CT suggested a diagnosis of an intrabdominal cystic. She had laparotomy and was offered my-
omectomies on account of a large subserous uterine mass arising from the right side of the uterine fundus, small subserous
fundal mass, intramural mass in the left side of the fundus and a cervical mass. Histology confirmed multiple uterine
leiomyomas with extensive cystic degenerative changes of the large subserous uterine myoma and adenomyosis of the left
fundal mass. Detecting the continuity of an abdominal mass even with extensive degenerative changes mimicking a cyst in
continuity with the uterus by a pedicle sign on imaging in the absence of ascites should arouse the diagnosis of pedunculated
subserosal leiomyoma. This should be further heightened when it is found in association with cervical myoma. Subserous
uterine leiomyoma should be considered in a patient of childbearing age with a grossly distended abdomen without obvious
evidence of pregnancy or malignancy. Large subserous uterine leiomyoma in an intraabdominal location may present with
diagnostic and surgical challenges that require interdisciplinary cooperation.
Keywords
Large subserous leiomyoma, multiple leiomyomas, adenomyosis, uterus, abdominal swelling
Introduction
Leiomyomas or fibroids are the most common benign
gynecological tumours that arise from the overgrowth of 1
Department of Surgery, University of Calabar/University of Calabar
smooth muscle and connective tissue of the uterus.1,2 Giant Teaching Hospital, Calabar, Nigeria
2
uterine leiomyomas are rare. It is described as giant when it Department of Obstetrics and Gynaecology, University of Calabar/
University of Calabar Teaching Hospital, Calabar, Nigeria
weighs 11.4 kg (25 pounds) or above.3,4 The largest re- 3
Department of Pathology, University of Calabar/University of Calabar
moved leiomyoma weighed 63.3 kg at post mortem in Teaching Hospital, Calabar, Nigeria
1888 and 45.4 kg from a person who survived.5 It is es- 4
Department of Surgery, University of Calabar Teaching Hospital, Calabar,
timated that less than 100 cases of giant uterine leiomyomas Nigeria
had been documented in literature.5 The prevalence of
Corresponding author:
uterine leiomyoma is increased during reproductive phase John A Ashindoitiang, Department of Surgery, University of Calabar
of life and rapidly decrease following menopause and Teaching Hospital, UNICAL Hotel Road, Calabar 540281, Nigeria.
highlighted the importance of hormonal factors as the cause Email: [email protected]
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2 Rare Tumors
for the neoplasm.5,6 However, the underlying pathogenesis jaundice, urinary symptoms nor family history of similar
of their development remains unclear with several risk swelling. Patient had dyspeptic symptoms for about 5 years.
factors such as positive family history, genetic alterations She had appendicectomy 12 years ago. She’s Para 0 + 2
and lifestyle factors identified.2 (termination of pregnancies), noticed some episodes of
Pedunculated uterine leiomyoma occurs when the mass scanty periods and shortened period of her menstrual flow
is in continuity with the uterus with a stalk and may grow (2 days). Her LMP was mid-August 2023.
either within the uterine cavity (submucosal) or outside of Examination showed a young woman otherwise
the uterus (subserosal) and the later may simulate ovarian healthy looking, not pale, afebrile, anicteric and no pedal
neoplasms or intraabdominal tumours.1,7 Leiomyomas oedema. Chest was clinically clear. The abdomen was
usually cause symptoms of heavy menstrual period, in- asymmetrically distended with a swelling that was esti-
fertility, abdominal distension and pressure symptoms that mated to have a fundal height of 32 weeks in the right half
may result in sensation of bowel distension, increased of the abdomen and extended across the midline,
urinary frequency and respiratory difficulty.3,4 Figure 1(a) and (b). There was an oblique scar in the right
They are easily recognized on imaging except with iliac fossa from appendectomy Figure 1(a). Swelling was
atypical presentation caused by degenerative changes.1 spherical, measured 30 cm × 25 cm. It was non-tender,
Definitive diagnosis was by histology that showed prolif- was possible to get above and below the swelling that was
eration of smooth muscle cells without atypia.1,3 Surgery is firm, smooth, mobile and not attached to overlying skin.
the treatment of choice for giant uterine leiomyoma.4 There was no demonstrable ascites and rectal examina-
Presented is a 28-year-old with progressive abdominal tion was unremarkable. A clinical diagnosis of an in-
swelling over 9 months duration. She was seen at the traabdominal mass was made.
surgical outpatient department on account of aesthetic Work up included full blood count (FBC), urea, elec-
displeasure that arose from an intraabdominal swelling trolytes and creatine (EUCr), clotting profile that were
without pregnancy. Following surgery, a definitive diag- unremarkable. Urinalysis was normal. Abdominopelvic CT
nosis of multiple leiomyoma with a large subserous uterine scan presented in the clinic showed a huge loculated cystic
leiomyoma with extensive cystic degeneration. We present a hypodense intrabdominal mass with thick enhancing wall
28-year old woman with a large subserous uterine leio- occupying predominantly the right flank and measured
myoma that presented as an intraabdominal tumor to 18.4 cm, Figure 2(a). It displaced the abdominal aorta and
highlight the diagnostic challenges that may arise. IVC to the left. Uterus was reported as bulky with AP
dimension of 8 cm showing a calcified myoma (5.0 ×
5.5 cm) in its myometrium that compressed the adjacent
Case report
bladder, Figure 2(b). The impression was: intra-abdominal
A 28-year-old woman presented to our facility in October mass with calcified uterine fibroid. There was no gyneco-
2023 with abdominal swelling in the past 9 months. It was logical consultation in the preoperative period.
insidious in onset, first noticed from her right flank and it During operative laparotomy the gynecologist was in-
progressively increased in size extending to the upper ab- vited with the following findings:
domen more on the right extending to the left. Swelling was
painless and there was no swelling elsewhere in her body. 1. Large subserosal pedunculated spherical uterine
There was no history of anorexia, vomiting, easy satiety, mass, FIGO type 7 that measured about 25 cm in
change in bowel habit, passage of dark tarry/blood in stool the widest dimention that arose from the right side of
nor weight loss. Furthermore, there was no history of the fundus of the uterus, Figure 3(a) and (b).
Figure 1. a, b- Clinical photographs showing abdominal distension, (a)- right lateral view in addition showed a post appendectomy scar
on the right lower abdomen. (b), from the foot of the bed showing asymmetrical distension, more on the right side.
Ashindoitiang et al. 3
Figure 2. a, b- CT- Axial section showing intraabdominal mass with degenerative changes (red arrow), (b)-CT- Sagittal section showing
abdominopelvic mass with “ultrashort pedicle sign” (blue arrows), dystrophic calcification of cervical myoma (brown arrow) and cystic
degenerative changes (red arrow).
Figure 3. (a)- Large subserous uterine leiomyoma insitu, (b)- Intraoperative, demonstrating the ultra-short pedicle.
2. Spherical subserosal uterine mass (that measured The abdomen was accessed through a midline incision
3.5 cm in the widest dimention) attached to the extending from the xiphisternum to the suprapubic area with
supero-anterior aspect of the uterus, Figure 4(a). hemostatic control. A huge mass was delivered that revealed
3. Intra-uterine mass located at the anterior part of the a large subserous uterine mass with an ultra-short peduncle
fundus, Figure 4(b). attached to the right side of the uterine fundus, FIGO 7,
4. Large spherical intra-cervical mass that measured Figure 3(a) and (b). Another relatively smaller subserous
about 10 cm in its widest dimention, Figure 4(b) masses was also noted, Figure 4(a). No adhesions were
5. Normal adnexa, liver, spleen, mesentery and no noted. The serosa was deroofed at the peduncle and the mass
ascites. was excised between two myometrial clamps. The my-
ometrial tissue was transfixed with polyglactin size two and
the serosal cover restored. The Gynecologist who was in-
Patient in supine position, under general anaesthesia vited discovered and removed another uterine mass located
with endotracheal intubation, nasogastric tube was passed at the cervix, Figure 4(b), alongside another subserous mass,
to deflate the stomach, a urethral catheter inserted to 4a. There was associated focal adenomyosis in the left
empty the urinary bladder/monitor hourly urinary output. fundal area also excised, (Figure 4(b)-part held between the
4 Rare Tumors
thumb and fingers). Mass closure was with polyamide two largest measured 10.5 cm and smallest 0.5 cm across the
monofilament. Skin was apposed with polyamide 2/0 in- widest dimensions, Figure 6. Microscopy revealed: uterine
terrupted vertical mattress sutures and the harvested spec- leiomyoma that showed bundles of smooth muscle fibers
imens sent for histology, Figure 5(a) and (b). Estimated that were haphazardly arranged. Individual muscle fibers
blood loss was 350 mls. Post operative period was un- were spindle shaped with bipolar eosinophilic cytoplasm
eventful and patient discharged. with cigar shaped nuclei. There were areas that showed
Histology was reported as follows: macroscopy-four hyalinization while the other areas showed cystic spaces and
different specimens, largest pedunculated subserous, cer- there was no atypia. Furthermore, this uterine leiomyoma
vical, left fundal and subserous masses that measured 18 × showed degenerative changes (hyaline and cystic),
17 × 13 cm, 11 × 9 × 5 cm, 3.5 × 3 × 2 cm and 3.5 × 2.5 × Figure 7(a) and (b). Histology of the subserosal and cervical
0.5 cm respectively. Cut surface of the largest mass (sub- intrauterine masses showed, interlacing bundles of smooth
serous uterine mass) showed multiple cystic spaces, the muscles fibers that were haphazardly arranged, individual
Figure 4. (a)- Multiple subserous leiomyomas (blue arrows), right ovary (yellow arrow), (b)- cervical leiomyoma (blue arrow) and
adenomyosis (green arrow).
Figure 5. (a)- Gross picture of subserous uterine leiomyoma- site the attachment of the pedicle (green arrow). (b)- Gross picture of
subserous uterine leiomyoma (red arrow), Leiomyoma of the uterine cervix (blue arrow).
Ashindoitiang et al. 5
Figure 6. Cut surface of intraabdominal subserous uterine leiomyoma, showing multiple cystic spaces, largest 10.5 cm in the widest
dimension and the smallest 0.5 cm.
Figure 7. a, b- Uterine leiomyoma. (a)- Histology of intraabdominal subserous uterine leiomyoma showed bundles of smooth muscle
fibers that were haphazardly arranged, individual muscle fibers were spindle shaped with bipolar eosinophilic cytoplasm with cigar
shaped nuclei. (b)- showed hyaline degeneration. There was no atypia, (c)- Uterine adenomyosis, blue arrow-endometrial glands, red
arrow-endometrial stroma, yellow arrow-myometrium.
6 Rare Tumors
muscle fibers were spindle shaped with bipolar eosinophilic pathogenesis of leiomyoma. MED 12 negative genotype is
cytoplasm with cigar shaped nuclei with no atypia- associated with larger fibroids.5 Mutations in the gene
subserous and uterine leiomyomata. Left intrauterine fun- encoding fumarase hydratase were shown to predispose
dal mass showed, sections of the endometrial glands and women to multiple leiomyomas.3
stroma deep in the myometrium of smooth muscle fibers The position of the leiomyoma in relation to the uterus
that were haphazardly arranged. The individual muscle determined the patients symptoms and diagnostic speci-
fibers were spindle shaped with bipolar eosinophilic cy- ficity.2 The most common location is the body of the uterus
toplasm with cigar shaped nuclei-uterine adenomyosis, and may also involve the uterine cervix in minority of
Figure 7(c). The definitive diagnoses were multiple uterine instances,1 our patient presented with multiple leiomyomas
leiomyomas and adenomyosis. She was referred to the of the corpus and cervix. Based on their positions within the
gynaecolosist for further care. uterine wall, leiomyomas were classified as: (a) intramural
(70%), (b) uterine cavity (10%)- submucosal, pedunculated
submucosal or pedunculated vaginal, (c) growing outwards
Discussion`
from the uterus (20%), further classified as cervical, sub-
Leiomyomata of the uterus are gynecological problems in serous, intraligamentous or pedunculated subserous (ab-
women of reproductive age with negative impact on their dominal).8 In a similar classification, it was divided into
health.3 They are the most common form of benign tumors extra-uterine and uterine fibroids which is further classified
of the female reproductive tract.1 However, giant uterine into intramural/interstitial (75%), submucosal (5%), sub-
leiomyoma are rare with about 100 cases documented serosal (10%) and cervical (1%).5 In the submucosal
worldwide.3 Pedunculated subserous uterine tumor in the (uterine cavity) or subserosal (abdominal) they may have a
abdominal location can attain a giant size with diagnostic connecting stalk (pedunculation).2 The above locations
and surgical challenges especially with nonspecific symp- determined the pattern of clinical presentation. Clinical
toms as was the experience with our patient. She presented presentation in majority of cases may be asymptomatic. The
to the surgical outpatient mainly on the account of the symptomatic women were most likely to present with
aesthetic discomfort occasioned by the increasing abdom- uterine bleeding: heavy menstrual period and frequent
inal girth in a non-pregnant state. Uterine leiomyoma is menstruation.2 In addition, frequent symptoms include
more common in nulliparous women and prevalent in black dyspareunia or chronic acyclic pain, they can affect fertility
women compared with the Caucasians, Asians or Hispanic with remarkable psychological impact on a woman’s life.2
women.3 It occurred in 20%–30% of females older than Our patient had amenorrhea of about 2 months, this should
30 years. At the age of 50 years, 80% of African and about have prompted a pregnancy test.
70% of Caucasian women had uterine leiomyoma.2 In Uterine leiomyoma has been described as giant based on
another study, it was estimated that by the 5th decade, as the weight of 11.4 kg or greater or have a diameter greater
many as 50% of women of African descent had than 17 cm or dimension of 33 × 28 ×22 cm.9 Due to the
leiomyoma.3 ability of the anterior abdominal wall become distended and
The etiology of uterine leiomyoma remain unknown the large volume of the abdominal cavity, uterine leio-
with several risk factors identified: positive family history, myoma (especially the subserous pedunculated) can grow
ovarian hormones, genetic mutations and lifestyle related into extremely large dimensions.3 This can result in a
factors. The later included diet, obesity, medical contra- feeling of dragging sensation of the abdomen resulting in
ception, smoking and exercise. The major hormones im- aesthetic displeasure from increased abdominal girth in
plicated in the growth of leiomyoma were ovarian steroids keeping with the experience of our patient. As the tumor
(estradiol and progesterone).2 The population of hormone grows, myomas can result in complications from com-
receptors have been known to be increased in fibroids.3 The pression related symptoms of dyspnea, frequent urination,
incidence of fibroid is decreased with prolonged use of oral bowel complaints2,3 and other unpredictable events as
contraceptive pill as well as increased number of term massive bleeding, adhesions to surrounding organs in-
pregnancies.3 The growth of leiomyoma appeared to be cluding displacement of organs/adjacent structures.4 The
dependent on the hormone estrogen. If a woman with index patient demonstrated hydroureters as reported in the
leiomyoma is still menstruating, the tumour will probably CT scan, the patient was however asymptomatic, reported
continue to grow slowly.7 Progesterone down-regulates also, was the displacement of the descending aorta and the
apoptosis in the tumor while estrogen increase produc- inferior vena cava (IVC). Kim et al reported a pedunculated
tion of extracellular matrix.3 Cytogenetic abnormalities leiomyoma in an umbilical hernia in a non-pregnant woman
occur in 50% of fibroids, most commonly, translocation with a large intra-abdominal lesion.10 Pedunculated sub-
within or deletion of chromosome, translocation of chro- serous myomas may result in an emergency arising from the
mosome 12 and 14 and occasionally structural aberrations complication of torsion.2 Leiomyoma may occur with en-
of chromosomes.3 MED 12 gene is associated in the dometriosis and adenomyosis with overlapping symptoms.2
Ashindoitiang et al. 7
As uterine leiomyoma outgrow their blood supply, com- standard.13 Abdominal hysterectomy is, however the most
plications may arise resulting in degeneration: hyaline, red, effective surgical therapy for giant uterine leiomyoma ex-
myxoid or cystic changes and dystrophic calcification.1,5 cept when pedunculated or when fertility is an issue and this
Rarely uterine leiomyoma may undergo malignant degen- should be discussed with the patient.3 Giant uterine leio-
eration resulting in sarcoma in less than 1.0% with some myoma cannot be treated with minimally invasive surgery.2
reports estimated to be as low as 0.2%.1,2,5 Uterine leio- Complications of surgery include massive blood loss
myomas have been misdiagnosed as adenomyosis, hema- and others like injury to adjoining structures occasioned
tometra, uterine sarcoma, ovarian masses, pregnancy and by adhesions/displacement to surrounding organs and
gastrointestinal tumours especially gastrointestinal stomal infection.4,12 Jonas et al reported a perioperative mortality
tumours (GIST).1,2,11 of 14.8%–16.7% among patients with giant uterine leio-
Preoperative imaging studies are useful to determine the myoma.4 Follow up of patients is aimed at the detection of
extent of the tumor and ultrasound sonography (USS) is the recurrence. Follow up of patients is for the detection of the
preferred modality of initial evaluation as it is easily recurrence of myomas and this will commence within 2–
available, non-invasive and convenient cost-benefit ratio. 6 weeks after surgery. Ultrasonography and pelvic exam-
An USS especially vaginal one can help to assess the mass, ination are commenced. Subsequent visits are scheduled for
vascularity, pedicle, adenxa, ascites and kidneys.1,2 This 3 months, 6 months and 1 year. If there is no recurrence in a
was a significant diagnostic deficiency in our diagnostic year, annual examinations are likely to be adequate.
work up. Uterine leiomyomas are often detected on CT and However, no studies have been done in support of this
may be incidental. Uterine leiomyoma and normal my- protocol.14 Prevention of massive uterine leiomyoma re-
ometrium are sometimes not distinguishable on CT except quires close surveillance and early surgical treatment.2
when they are calcific or necrotic changes.12 The CT of the Treatment of lifestyle-associated risk factors and vitamin
patient revealed a pedicle sign, cystic degenerative signs D supplementation, use of statin and dietary modifications
may create heterogenous or an unusual appearance that appears to be productive along with parity.2
contributed to the diagnostic challenges.1,11 Atypical ap-
pearance of leiomyoma limits its preoperative informative
value of some of the imaging modalities2 as was demon- Conclusion
strated in our patient with extensive degenerative changes.
MRI is more specific in the determination of the origin of In conclusion, large subserous uterine leiomyoma should be
uterine leiomyoma including degenerative changes. The considered in a patient of childbearing age with a grossly
multiplanar imaging may reveal a vascular pedicle or distended abdomen without obvious evidence of pregnancy
another form of attachment of uterine leiomyoma. Non- or malignancy. Detecting the continuity of an abdominal
degenerated leiomyoma shows characteristic low- mass (even with extensive cystic degenerative changes)
intermediate signal intensity on T1-W1 and low signal with the uterus by a stalk (pedicle sign) on imaging in the
intensity on T2-W1. Degenerated leiomyomas are variable absence of ascites should arouse the diagnosis of pedun-
in terms of MRI signal characteristics. Myxoid degeneration culated subserosal leiomyoma. Large subserous uterine
and necrosis may present as high signal intensity areas on leiomyoma may present a diagnostic and surgical challenge
T2-W1 without enhancement. Cobblestone-like tissue at- that require expertise and interdisciplinary cooperation. It
tributable to hyaline degeneration with foci of high signal can be successfully managed without complications with
intensity that represented areas of infarction caused by rapid proper diagnosis and surgical expertise.
growth seen on both T1 and T2-W1. No imaging modality
can exclude malignancy leaving the diagnosis of a giant Acknowledgements
uterine fibroid a challenge.2 Histologically, smooth muscle
We acknowledge the patient and all the participating authors for
cells proliferate, occasionally histologic composition may
their contribution to the paper.
not allow precise separation from intestinal organs.2 Di-
agnostic laparoscopy is valuable especially in the resolution
of diagnostic challenges and in some instances a preferred Declaration of conflicting interests
route for surgery.5 The author(s) declared no potential conflicts of interest with
Treatment is individualized based on the severity of respect to the research, authorship, and/or publication of this
symptoms and the need to preserve fertility.12 Surgical article.
options include myomectomy and abdominal hysterectomy,
the main indication for myomectomy is the preservation of
the uterus for childbearing in the younger-aged woman as Funding
was indicated in our patient.3 Robotic-assisted laparoscopic This research received no financial support for the research, au-
myomectomy (RALM) is the treatment of choice and gold thorship, and/or publication of this article.
8 Rare Tumors