Sushila Ladumor, MD

Case History: A 36-year-old male presented with weak stream and pain.

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Case History: 36-year-old male presented with weak stream and pain.

36 y/o Male, Weak Stream, Pain

 A 42 year-old male patient referred to rule out intestinal obstruction; history of appendectomy and laparotomy.

 There are surgical staples in the right hypochondriac region indicating previous resection side. There is obvious retroperitoneal predominately low density mass of about 12 cm x 8 cm with multiple cystic areas with the thick peripheral rim with anterior displacement of the second part of the duodenum and mesenteric vessels with distended stomach with persistence of oral contrast in gastric lumen even in delayed series suggestive of partial outlet obstruction Proximal small gut loops are distended and there is obvious narrowed segment in the distal small gut with associated bowel wall thickening in right lower quadrant, suggestive of distal intestinal obstruction.

Paragangliomas are rare tumors that arise from extra adrenal chromaffincells. During embryogenesis, neural crest cells migrate to diverse locations in the body and differentiate into chief and sustentacular cells, which are designated as paraganglia.

Extra luminal perigastric masses are frequently considered to be gastrointestinal stromal tumors (GISTs) or other soft tissue sarcomas, when histological confirmation is not possible. Paragangliomas may have a similar radiographic appearance. In the absence of histological diagnosis and symptoms of catecholamine excess, paragangliomas may be mistaken for GISTs. Perigastric abdominal mass presumed to be a GIST after nondiagnostic biopsy, is presented.

On abdominal CT, there are no unique imaging characteristics specific for paragangliomas. Consequently, these tumors may be mistaken for other primary epithelial or mesenchymal abdominal tumors. For instance, GIST, the most common sarcoma of the gastrointestinal tract, may have a similar radiographic appearance. They are often large (84 percent N 5 cm) tumors arising from the stomach with heterogeneous enhancement (84 percent) and central necrosis (37 percent). Retroperitoneal paragangliomas affect mainly adults in the fourth or fifth decade of life, with no sex predilection.

While size and location help to differentiate GISTs from paragangliomas, correlation with clinical symptoms is needed. A presumed GIST considered to be resectable does not necessarily warrant preoperative biopsy, and thus further diagnostic workup is not pursued.

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Paraganglioma

CASE 1: An elderly male patient with long term history of right inquinal swelling presented to emergency department with acute pain in abdomen along with nausea, vomiting, distension of abdomen and increase in swelling and tenderness in right inquinal region.

Frontal erect abdominal X-ray shows only two dilated air-fluid level on right side. Air and fecal matter in colon. In view of history CT scan of abdomen recommended.

Axial CT with contrast images of abdomen shows hugely distended stomach and dilated small bowel.

Continuous axial CT with contrast images of abdomen shows dilated small bowel with right inguinal hernia containing dilated mildly thick wall small bowel and some fluid.

Continuous axial CT with contrast images of abdomen shows right inguinal hernia containing dilated mildly thick wall small bowel and some fluid. Patient has penile prosthesis.

Coronal reformatted images of abdomen shows hugely distended stomach, dilated small bowel and right inguinal hernia containing dilated mildly thick wall small bowel and some fluid represent incarcerated hernia causing small bowel obstruction.

Saggital and coronal reformatted images of abdomen shows hugely distended stomach, dilated small bowel and right inguinal hernia containing dilated mildly thick wall small bowel and some fluid represent incarcerated hernia causing small bowel obstruction.

Findings represent incarcerated hernia. Emergency physician informed immediately for further management.

CASE 2: An elderly male patient with previous history of abdominal surgery and long term swelling at the site of surgical scar at left side of lower abdomen and now presented to emergency with acute abdominal pain and distension along with nausea, vomiting and increase in swelling with tenderness at the site of swelling.

Supine X-ray abdomen shows dilated small bowel indicating small bowel obstruction.

Erect X-ray abdomen shows multiple dilated air-fluid level in small bowel, indicating small bowel obstruction.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect, distal bowel loops are not dilated represent incarcerated hernia.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated represent incarcerated hernia.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated represent incarcerated hernia. Multiple non complicated colonic diverticulum.

Reformatted images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated, no pneumatosis, represent incarcerated hernia. Multiple non complicated colonic diverticulum.

Emergency physician informed immediately for further management.

Definition: A hernia is the protrusion of an organ or the fascia of an organ through the wall of the cavity that normally contains it. There are different kinds of hernia, each requiring a specific management or treatment.

Indirect hernia - An indirect inguinal hernia follows the tract through the inguinal canal. This results from a persistent process vaginalis. The inguinal canal begins in the intra-abdominal cavity at the internal inguinal ring, located approximately midway between the pubic symphysis and the anterior iliac spine. The canal courses down along the inguinal ligament to the external ring, located medial to the inferior epigastric arteries, subcutaneously and slightly above the pubic tubercle. Contents of this hernia then follow the tract of the testicle down into the scrotal sac.

Direct hernia - A direct inguinal hernia usually occurs due to a defect or weakness in the transversalis fascia area of the Hesselbach triangle. The triangle is defined inferiorly by the inguinal ligament, laterally by the inferior epigastric arteries, and medially by the conjoined tendon.

Femoral hernia - The femoral hernia follows the tract below the inguinal ligament through the femoral canal. The canal lies medial to the femoral vein and lateral to the lacunar (Gimbernat) ligament. Because femoral hernias protrude through such a small defined space, they frequently become incarcerated or strangulated.

Umbilical hernia - The umbilical hernia occurs through the umbilical fibromuscular ring, which usually obliterates by 2 years of age. They are congenital in origin and are repaired if they persist in children older than age 2 to 4 years.

Richter hernia - The Richter hernia occurs when only the antimesenteric border of the bowel herniates through the fascial defect. The Richter hernia involves only a portion of the circumference of the bowel. As such, the bowel may not be obstructed, even if the hernia is incarcerated or strangulated, and the patient may not present with vomiting. The Richter hernia can occur with any of the various abdominal hernias and is particularly dangerous, as a portion of strangulated bowel may be reduced unknowingly into the abdominal cavity, leading to perforation and peritonitis.

Incisional hernia - This iatrogenic hernia occurs in 2 percent to 10 percent of all abdominal operations secondary to breakdown of the fascial closure of prior surgery. Even after repair, recurrence rates approach 20 percent to 45 percent.

Spigelian hernia - This rare form of abdominal wall hernia occurs through a defect in the spigelian fascia, which is defined by the lateral edge of the rectus muscle at the semilunar line (costal arch to the pubic tubercle) The two subtypes are interstitial and subcutaneous, which are best defined using CT and assist with optimizing the surgical approach when indicated.

Obturator hernia - This hernia passes through the obturator foramen, following the path of the obturator nerves and muscles. Obturator hernias occur with a female-to-male ratio of 6:1, because of a gender-specific larger canal diameter and predominately in the elderly. Because of its anatomic position, this hernia presents more commonly as a bowel obstruction than as a protrusion of bowel contents.

Hernia symptoms and signs: The signs and symptoms of a hernia can range from noticing a painless lump to the severely painful, tender, swollen protrusion of tissue that you are unable to push back into theabdomen (an incarcerated strangulated hernia).

- It may appear as a new lump in the groin or other abdominal area.

- It may ache but is not tender when touched.

- Sometimes pain precedes the discovery of the lump.

- The lump increases in size when standing or when abdominal pressure is increased (such as coughing).

- It may be reduced (pushed back into the abdomen) unless very large.

- It may be an occasionally painful enlargement of a previously reducible hernia that cannot be returned into the abdominal cavity on its own or when you push it.

- Some may be chronic (occur over a long term) without pain.

- An irreducible hernia is also known as an incarcerated hernia.

- It can lead to strangulation (blood supply being cut off to tissue in the hernia).

- Signs and symptoms of bowel obstruction may occur, such as nausea and vomiting.

- This is an irreducible hernia in which the entrapped intestine has its blood supply cut off.

- Pain is always present, followed quickly by tenderness and sometimes symptoms of bowel obstruction (nausea and vomiting).

- The affected person may appear ill with or without fever.

- This condition is a surgical emergency.

Sushila Ladumor, MD, FRCR, consultant radiologist with multi-modality imaging experience, working in Medical Imaging Department, King Abdulaziz Medical City, Riyadh, Saudi Arabia

Incarcerated hernia: X-ray and CT scans evaluate inquinal swelling and and long-term swelling in elderly males.

Incarcerated Hernia

Clinical History: A 33-year-old female with abdominal distension and suspected ovarian mass.
CT scan of abdomen and pelvis done with oral and intravenous contrast with multiple axial sections along with coronal and sagittal reformation.

Findings: Large abdomino-pelvic cystic mass with thin septations. No enhancing nodule. Right ovary is seen and appears within normal limit. Features are suggestive of benign mucinous cyst adenoma of left ovary.

Multi-planner, multi-sequential MRI of abdomen and pelvis performed with and without contrast. Large multi-loculated cystic lesion arising from the left adnexa measuring maximum dimension of 30 x 11 x 27 cm in craniocaudal, AP, and mediolateral dimension.

Post gadolinium T1 images shows no enhancement of mass or septa.
 

The lesion demonstrated multiple internal thin septations. The mass exerts significant mass effect on the bladder and the uterus which is shifted to the left side. The right adnexum appears unremarkable. No free fluid identified in the pelvis. No obvious retroperitoneal lymph nodes seen. No obvious carcinomatosis identified. The appearance of the lesion is mostly in keeping with a benign epithelial ovarian neoplasm. Given the size of the mass and the presence of multiple septations, the appearance is most likely in keeping with mucinous cystadenoma.

Post-op pathological diagnosis: Mucinous cystadenoma.

Overview: Mucinous cystadenoma is a type of tumor in the cystadenoma grouping. There are four major categories of ovarian tumors:

1. Epithelial tumors (65 percent to 75 percent) - serous or mucinous cystadenoma/carcinoma, clear cell carcinoma, Brenner tumor
2. Germ cell tumors (15 percent) - dysgerminoma, embryonal cell cancer, choriocarcinoma, teratoma
3. Sex-chord-stromal tumors (5 percent to 10 percent) - granulosa cell tumor, thecoma, fibroma
4. Metastatic tumors (10 percent) - uterine, stomach, colon, breast, lymphoma

Mucinous cystadenomas make up 15 percent to 20 percent of all ovarian tumors. They often become very large and can extend up into the abdomen. Pseudomyxoma peritonei can result if the tumor ruptures and spills its contents into the abdomen.

These tumors are usually evaluated using ultrasound, CT scan, or MRI. Findings on imaging studies are nonspecific. These ovarian tumors are usually multi-septated, cystic masses with thin walls. They also contain varying amounts of solid tissue which consists of proliferating stromal tissue, papillae, or malignant tumor cells.

Mucinous cystadenomas are divided into three categories: benign, borderline, and malignant.
Survival is largely dependent on the histology of the tumor, with a 10-year survival rate of 100 percent for benign tumors, 60 percent for borderline tumors, and only 34 percent for the malignant subtype. There is some difference in ages of the peak incidence for the different subtypes with considerable overlap as described below. In general, benign tumors tend to present earlier, while malignant tumors are often seen later in life.

Benign mucinous cystadenomas compose 80 percent of mucinous ovarian tumors and 20 percent to 25 percent of benign ovarian tumors overall. The peak incidence occurs between 30 to 50 years of age. Benign tumors are bilateral in 5 percent to 10 percent of cases. Borderline mucinous cystadenomas make up about 10 percent of mucinous ovarian neoplasms and are bilateral in 10 percent of cases.

Malignant mucinous cystadenoms are rare, and encompass 10 percent of mucinous ovarian tumors and 5 percent to 10 percent of primary malignant ovarian neoplasms overall. They are bilateral in 15 percent to 30 percent of cases and have a peak incidence between 40 to 70 years of age. It can present in the ovary.

http://emedicine.medscape.com/article/255865-overview

http://www.annclinlabsci.org/content/33/4/465.abstract

http://journals.lww.com/intjgynpathology/Abstract/2005/01000/Mucinous_Tumors_of_the_Ovary__A_Review.3.aspx

http://en.wikipedia.org/wiki/Mucinous_cystadenoma

Mucinous cystadenom: 33-year-old female with abdominal distension and suspected ovarian mass. CT scan of abdomen and pelvis done with oral and intravenous contrast with multiple axial sections along with coronal and sagittal reformation.

Author | Sushila Ladumor, MD

Articles

36 y/o Male, Weak Stream, Pain

Paraganglioma

Incarcerated Hernia

Mucinous Cystadenoma