|Ahead of print publication
Primary small gut lymphoma presenting as an incarcerated inguinal hernia in an adult
Prosanta Kumar Bhattacharjee, Amitabha Sarkar, Somnath Biswas, Tanvi Goel
Department of Surgery, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
|Date of Submission||01-Jul-2018|
|Date of Decision||18-Sep-2018|
|Date of Acceptance||18-Nov-2018|
Prosanta Kumar Bhattacharjee,
Flat No. 5, 4th Floor, “Suryatoran Apartment” 114/A, Barasat Road, Kolkata - 700 110, West Bengal
Source of Support: None, Conflict of Interest: None
A 73-year-male presented with features of subacute small gut obstruction of 10 days duration. He also complained of a painless, slowly enlarging swelling in the right groin, which was first noticed 10 years ago. There was a history of weight loss, anorexia, and asthenia but no history of fever, respiratory, or urinary symptoms. He was a smoker and was on medication for hypertension and type 2 diabetes mellitus. The general health of the patient was poor. He appeared pale and dehydrated. No lymphadenopathy was evident on the general survey. The abdomen was distended, tense with hyperactive bowel sounds. Examination of the groin and genitalia revealed right inguinal hernia and an irreducible, firm, solid, nontender, 6 cm × 5 cm scrotal mass separate from the right testis. Digital rectal examination revealed no abnormality. Contrast-enhanced computed tomography of the abdomen showed dilated gut loops, right inguinal hernia, and a gut related endophytic soft-tissue mass in the scrotum. Exploration after resuscitation revealed a firm, endoluminal soft-tissue mass arising from the apex of the herniated loop of the small gut which was obliterating its lumen. The tumor-bearing segment of the gut was resected through a groin incision. We then performed a laparotomy to bring out the ends of the bowel loops as double barrel ileostomy. The hernial defect was then repaired. Postoperative recovery was uneventful. Histopathology of the excised specimen suggested the possibility of Non-Hodgkin's lymphoma of small gut. Immunohistochemistry confirmed it to be low-grade follicular B-cell NHL.
Keywords: Inguinal hernia, intestinal obstruction, non-Hodgkin's lymphoma, small intestine
|How to cite this URL:|
Bhattacharjee PK, Sarkar A, Biswas S, Goel T. Primary small gut lymphoma presenting as an incarcerated inguinal hernia in an adult. Formos J Surg [Epub ahead of print] [cited 2019 Feb 16]. Available from: http://www.e-fjs.org/preprintarticle.asp?id=250873
| Introduction|| |
Small-bowel tumors are rare and account for <2% of all gastrointestinal (GI) neoplasms. Majority involves the duodenum. Primary jejuno-ileal tumors are rarer still. They may be adenocarcinoma, GI stromal tumor (GIST), or rarely lymphoma which either presents late with vague nonspecific symptoms, sometimes detected incidentally during surgery for some other conditions or the presentation may be with intestinal obstruction. Primary B-cell non-Hodgkin's lymphoma (NHL) of small bowel makes up roughly 20% of small bowel tumors.
Half of the patient with symptomatic small bowel tumors presents with intestinal obstruction, while the rest either with a palpable abdominal mass, GI hemorrhage, or perforation., There are few reports of a GIST of the small intestine presenting either with a complicated inguinal hernia, or as a scrotal swelling mimicking an inguinal hernia., A rare case of lymphoma of the hernia sac detected on routine microscopic examination of the excised sac has also been reported. However, an extensive search of the published literature revealed no report of a low-grade primary follicular B-cell variety of small bowel NHL presenting with obstructed inguinal hernia.
We present a successfully managed unique case of lymphoma of the small gut presenting with scrotal swelling and causing obstruction of an inguinal hernia in a 73-year-man.
| Case Report|| |
A 73-year-male presented with features of subacute small gut obstruction of 10 days duration along with a gradually increasing painless swelling in the right side of scrotum for over 10 years. The scrotal swelling did not increase in its size with physical activities, nor did it ever reduce spontaneously on lying down.
There was a history of significant weight loss, anorexia, asthenia, loss of appetite, and fatigability over the last 8 months. History of fever, hematemesis, melena, or bleeding per rectum were absent. Similar earlier history of abdominal pain, distension, and vomiting or any other significant history in the past were absent. He was a smoker (15 pack-years), and on medications for hypertension and type 2 diabetes mellitus. Family history was negative for any malignancy.
On examination, he appeared dehydrated, pale, and of poor nutritional status. BMI was 18, Karnofsky score was 40 and Eastern Cooperative Oncology Group score 3. We could not detect any cervical or inguinal lymphadenopathy.
The abdomen was symmetrically distended. Prominent bowel loops arranged in a stepladder fashion and showing visible peristalsis could be seen centrally. The abdomen felt tense with no localized tenderness, guarding, or rigidity. No organomegaly or abdominal lump was palpable. There was expansile cough impulse at the right root of the scrotum and a non-tender, firm, solid, 6 cm × 5 cm, irreducible and nontrans illuminant scrotal mass palpable separately from the testis. Peristaltic sounds were hyperactive. Digital rectal examination revealed no abnormality.
Positive findings on routine hemogram, blood biochemical assays, serology, and chest x-ray were anemia, minor electrolyte imbalance, and hyperglycemia. Contrast-enhanced computed tomography (CECT) of the abdomen showed dilated gut loops, right inguinal hernia, and a gut related endoluminal soft-tissue scrotal lesion on the right [Figure 1]. We planned an emergency exploration after the correction of hydration and glycemic status with the provision of two units of the packed red blood cell for transfusion.
|Figure 1: Contrast-enhanced computed tomography showed massive dilatation of gut loops, right inguinal hernia with a gut related soft tissue mass within the scrotum|
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Exploration, through a groin incision, revealed a firm, endophytic, lumen occluding soft tissue mass at the apex of the herniated small gut loop. The gut proximal to the mass was dilated and the superficial inguinal ring was not causing any occlusion of the gut lumen.
Approximately 17 cm of the tumor-bearing segment of the small gut was resected and the ends of the bowel were brought out as double barrel ileostomy after laparotomy. We then repaired the hernial defect. The cut open specimen showed a submucosal fleshy tumor measuring 6.4 cm × 5 cm × 2.4 cm [Figure 2]. Adjoining mesentery had a small lymph node.
|Figure 2: Cut open view of the resected specimen showing the endophytic soft tissue mass involving the gut wall|
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Post-operative recovery was uneventful.
Tumor histopathology showed sheets of atypical lymphoid cells with opened up nuclear chromatin, distinct nucleoli, and a fair number of mitotic figures, compatible with the diagnosis of NHL [Figure 3]. The infiltration was up to the subserosa and there were areas of necrosis. Resection margins and lymph node were free of tumor.
|Figure 3: Photomicrograph showing large, mature lymphocyte, with scanty cytoplasm, irregular nucleus and dense chromatin (H and E, ×40)|
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Immunophenotyping confirmed it to be low-grade follicular B-cell NHL, being positive for B-cell associated antigens CD20 [Figure 4].
|Figure 4: Immunohistochemistry image showing CD20 positivity of lymphoid follicles of diffuse, large B cell lymphoma (immunohistochemistry, ×100)|
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We referred the patient to the oncology department of our hospital where he has not been advised any adjuvant therapy in view of the localized, low-grade nature of the disease and its complete surgical removal. They counseled him to follow-up regularly in their outpatient department.
| Discussion|| |
An inguinal hernia sac may contain omentum, small, or large intestine, appendix, Meckel's diverticulum or urinary bladder. Rarely, the sac may harbor a malignancy. Authors have reported GIST arising from the small intestinal loop within the hernial sac, lymphoma of the hernia sac, and spermatic cord.,, Literature search by us could retrieve only one case of NHL of the sigmoid colon as part of a sliding hernia, but no case of primary small gut NHL presenting with features of obstructed inguinal hernia.
NHL, mostly of B-cell origin, may involve the GIT either as part of a generalized disease or rarely arise primarily from the gut; the distal small intestine is most commonly affected owing to its large amount of sub-mucosal lymphoid tissue (Peyer's patches). The World Health Organization has cited primary intestinal follicular lymphoma as a variant of follicular lymphoma. The GIT is the most common site of extranodal involvement, others being skin, eyes, breasts, and testes.
The common presentations of gut NHL are weight loss and pain abdomen; at a later stage, intestinal obstruction or rarely perforations have been reported. The prognosis of NHL depends on the stage at diagnosis, cell lineage, its grade, patient's age and presence of 'B' symptoms (fever, weight loss, and night sweats). Primary B-cell low-grade follicular NHL usually runs an indolent course while systemic follicular lymphoma with secondary GI involvement may exhibit systemic symptoms.
Our patient had the tumor in the herniated small gut for more than a decade. With an increase in its size, it became irreducible, and later intestinal obstruction supervened following occlusion of the gut lumen. We established the primary nature of the NHL based on lack of evidence of lymphoma on peripheral blood smear, chest X-ray, and CECT. En-block resection ensuring tumor-free margins, as we did, is the treatment of choice for complications such as obstruction, hemorrhage, or perforation.
Surgical resection is the mainstay of treatment for localized primary lymphomas. Experts consider surgery alone as an adequate treatment for patients with early and localized low-grade NHL disease; however, systemic chemotherapy becomes essential when patients undergo surgical debulking.
Considering the age of our patient and the fact he underwent complete removal of the tumor, the oncologists considered surgery alone to be adequate.
| Conclusions|| |
NHL of the small gut may be considered as a very rare differential diagnosis of inguinoscrotal swelling in adults when other common causes have been excluded.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Ito H, Perez A, Brooks DC, Osteen RT, Zinner MJ, Moore FD Jr, et al.
Surgical treatment of small bowel cancer: A 20-year single institution experience. J Gastrointest Surg 2003;7:925-30.
Yang YS, Huang QY, Wang WF, Sun G, Peng LH. Primary jejunoileal neoplasmas: A review of 60 cases. World J Gastroenterol 2003;9:862-4.
Beltran MA, Cruces KS. Primary tumors of jejunum and ileum as a cause of intestinal obstruction: A case control study. Int J Surg 2007;5:183-91.
Tinoco-González J. Gastrointestinal stromal tumor (GIST) presenting as a groin mass mimicking and incarcerated hernia. Int J Surg Case Rep 2015;6C: 166-8.
Mulla MG, Yeung J, Reynolds JR. Gastrointestinal stromal tumor (GIST) mimicking an indirect inguinal hernia, a rare presentation. Eur Surg 2007;39:270-2.
Goyal A, Mansel RE, Goyal S. Gastrointestinal stromal tumor in an inguinal hernial sac: An unusual presentation. Postgrad Med J 2003;79:707-8.
Veal DR, Hammil CW, Wong LL. Lymphoma diagnosed at inguinal hernia repair. Hawaii Med J 2010;69:32-4.
Saha TK, Hakim HA, Banik SK, Haque MR, Chowdhury TN, Talukdaer MT. Non-hodgkin's lymphoma in herniated sigmoid colon presenting as an irreducible left sided inguinal hernia – A case report and review of the literature. J Dhaka Med Coll 2015;24:73-5.
Harris NL, Swerdlow SH, Jaffe ES, Ott G, Nathwani BN, Jong D, et al
. Follicular lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al
, editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC; 2008. p. 220-6.
Iwamuro M, Kondo E, Takata K, Yoshino T, Okada H. Diagnosis of follicular lymphoma of the gastrointestinal tract: A better initial diagnostic workup. World J Gastroenterol 2016;22:1674-83.
Tondini C, Giardini R, Bozzetti F, Valagussa P, Santoro A, Bertulli R, et al.
Combined modality treatment for primary gastrointestinal non-hodgkin's lymphoma: The milan cancer institute experience. Ann Oncol 1993;4:831-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]