Laparoscopic Management of an Obstructing ... - Springer Link

Surg Today (2009) 39:606–609 DOI 10.1007/s00595-007-3807-y

Laparoscopic Management of an Obstructing Granulocytic Sarcoma of the Jejunum Causing Intussusception in a Nonleukemic Patient: Report of a Case CHINNUSAMY PALANIVELU, MUTHUKUMARAN RANGARAJAN, RAMAKRISHNAN SENTHILKUMAR, and SHANKAR ANNAPOORNI GEM Hospital & Postgraduate Institute, 45-A Pankaja Mill Road, Ramnathapuram, Coimbatore 641045, India

Abstract Granulocytic sarcoma is an extramedullary tumor of immature myeloid cells which is often a forerunner to the development of acute myelogenous leukemia. Granulocytic sarcoma of the gastrointestinal tract frequently involves the small intestine and often presents with abdominal pain and obstruction. Our patient presented with a proximal jejunal mass causing intussusception and obstruction. This type of manifestation has never before been reported. A laparoscopy-assisted resection of the affected portion of jejunum was performed for him. The initial pathological findings were high-grade non-Hodgkin’s lymphoma; immunohistochemistry confirmed a diagnosis of granulocytic sarcoma. After a follow-up of 14 months, there was no evidence of leukemia. This condition is often mistaken for lymphoma and confirmation is necessary by immunohistochemistry. Chemotherapy is the treatment of choice and surgery is indicated only in the event of complications, such as bowel obstruction, bleeding, or perforation. The prognosis of granulocytic sarcoma is similar to that of myeloid leukemia. Key words Jejunal tumor · Granulocytic sarcoma · Laparoscopy · Resection

Introduction This tumor was first described as a chloroma by Burns in 1811.1 Rappaport renamed it “granulocytic sarcoma” in 1966, because not all of the cells are green; 30% are white, gray, or brown, depending on the state of oxidation of the pigmented enzyme or the different cellular enzyme concentrations.2 Granulocytic sarcoma (GS) Reprint requests to: M. Rangarajan Received: April 2, 2007 / Accepted: October 21, 2007

was first linked to myeloid neoplasms in 1893 by Dock.3 Isolated involvement of the gastrointestinal tract is uncommon. In this article, we report a patient with a GS of the proximal jejunum causing intussusception and obstruction. This type of presentation of granulocytic sarcoma has not yet been previously reported in the literature.

Case Report The patient was a 52-year-old man who presented in the emergency room with complaints of abdominal distention and pain that had recently been increasing in severity. He also experienced bilious vomiting for the previous 5 days. The pain was constant and present in all abdominal regions. He was afebrile, anemic, and dehydrated. Blood pressure was 100/70 mmHg and pulse rate 105 beats/min. A routine blood examination revealed an electrolyte imbalance. On physical examination, there was upper abdominal distension. Plain abdominal radiogram showed dilated loop of proximal jejunum with fluid levels. Upper gastrointestinal endoscopy was performed, which showed a tumor in the third part of the duodenum with intussusception (Fig. 1). A computed tomography (CT) scan revealed a welldefined hypoechoic lesion arising from the proximal jejunum causing total obstruction (Fig. 2). A nasogastric tube was placed; dehydration and electrolyte imbalance were corrected. A clinical diagnosis of subacute intestinal obstruction was made and the patient was scheduled to undergo diagnostic laparoscopy. Pneumoperitoneum was achieved via a Veress needle and pressure maintained at 12 mmHg. The 30° laparoscope was introduced through a 10-mm umbilical port. The other two 5-mm ports were placed in both the right and left lumbar areas. The surgeon stood between the legs facing the monitor located at the head-end of the patient. The perioperative findings consisted of a dilated proximal

C. Palanivelu et al.: Laparoscopy for Jejunal Granulocytic Sarcoma

Fig. 1. An endoscopic view of the mass intussuscepted into the duodenum

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Fig. 3. The appearance of a tumor on laparoscopy

Fig. 4. The lesion with the jejunum exteriorized via a minilaparotomy

Fig. 2. A computed tomography scan showing a tumor in the proximal jejunum (arrows)

jejunal, a tumor about 15 cm from the duodenojejunal flexure, with a collapsed jejunum distal to the tumor (Fig. 3). The tumor was seen to extend to the adjoining mesentery. A minilaparotomy measuring 4 cm in size was made in such a way as to include the umbilical port site, and the lesion was thereafter resected (Fig. 4). The mesentery was mobilized with Ultracision shears and

the tumor along with 3 cm of normal jejunum on either side was resected. An end-to-end anastomosis was performed using 000 vicryl in a single layer. After completion, the bowel was returned to the peritoneal cavity. The minilaparotomy wound was closed in two layers. The total operating time was 82 min and there was no blood loss. The ingestion of liquids was commenced orally on the 3rd postoperative day (POD) after the patient had a bowel movement, followed by soft diet on the 4th POD, and he was discharged on the 5th POD. There were no postoperative complications. The gross pathology showed a 6-cm mass, which was a homogeneous, fleshy, and transmural lesion, extending to the adjoining mesentery. All the margins of the specimen were negative for the tumor. Histopathology showed a diffuse, transmural infiltrate of lymphoid cells bordered

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C. Palanivelu et al.: Laparoscopy for Jejunal Granulocytic Sarcoma

Fig. 5. Histopathology: diffuse, transmural infiltrate of lymphoid cells, prominent mitotic figures, few eosinophilic precursors, and scant cytoplasm (left ×40; right ×100)

Fig. 6. A CD43 positivity can be seen as a dark brown stain on the cell surface (×40). B MPO positivity is visualized as a darkly stained cytoplasm of tumor cells (×10)

by normal mucosa (Fig. 5). At higher magnification, the cells had prominent mitotic figures, few eosinophilic precursors, and scant cytoplasm. On immunohistochemical staining, however, the cells were found to be negative for CD3, CD19, and CD20, and positive for MPO (Fig. 6A) and CD43 (Fig. 6B). Since the final diagnosis was now granulocytic sarcoma, we investigated the patient for leukemia. A bone marrow biopsy (normal erythroid : myeloid ratio, absence of atypical cells, and all cells showing normal lineage of maturation) revealed no evidence of leukemia. The patient was followed up at 1, 3, 6, and 14 months following surgery, and so far he has demonstrated no evidence of leukemia.

Discussion Granulocytic sarcoma is a rare occurrence with an estimated incidence of 0.7 per million in children and 2 per million in adults.4 This tumor occurs in about 5% of myeloid leukemias in adults and 13% of children.5 Previous terms used to describe this entity include extramedullary myeloid tumor, GS, chloroma, and chloroleukemia. These tumors can develop in patients with acute myeloid leukemia (AML) or in the accelerated phase of chronic myeloleukemia and rarely, they may be the first manifestation of AML, preceding the onset in marrow and blood by months or years.6 Granu-

locytic sarcoma has been reported to occur in just about every anatomic location imaginable. The most common sites are the skin (13%–22%), bone/spine (9%–25%), and lymph nodes (15%–25%).7 A number of cases of GS involving the small intestine have been reported in the literature, though isolated lesions in the gastrointestinal tract are extremely rare. The patients may present with epigastric pain, chronic anemia, and gastrointestinal bleeding. A perforation and small bowel obstruction have also been reported. Indeed, our patient presented with symptoms of gastric outlet obstruction as the tumor was obstructing the jejunal lumen, close to the duodenojejunal flexure. The most frequently involved region of the gastrointestinal tract is the small intestine (10%).8 Other regions of the gastrointestinal tract that have been reported to be involved are the stomach, large intestine, and appendix (only four cases involving the appendix have been reported).9 In a large study, the majority of reported cases presented as isolated masses (69%), whereas in patients with multiple lesions, up to four organ systems involved at one time were described.10 The majority of the patients with GS tend to have leukemia at presentation, or they will eventually develop leukemia. Our patient has been followed up for 14 months now, with no evidence of leukemia as yet. A large-scale retrospective study by Imrie et al. reported the median time for patients with isolated GS to develop AML to be about 9 months and the median survival to be about 22 months.11

C. Palanivelu et al.: Laparoscopy for Jejunal Granulocytic Sarcoma

It is imperative to differentiate GS from lymphomas, as the treatment is significantly different from that of malignant lymphoma. The diagnosis of GS may be difficult even on histopathology, and indeed the incidence of a misdiagnosis may be as high as 40%.12 Immunohistochemistry (IHC) staining is confirmatory, and tumor tissue will show positivity for MPO, CD3, CD19, and CD20. In our patient, histopathology alone could not diagnose GC, whereas IHC did. Granulocytic sarcoma has been treated with systemic chemotherapy, a surgical resection, radiation therapy, and peripheral stem cell/bone marrow transplantation. Surgery is not usually the first option, and it is only indicated in the event of complications like intestinal obstruction or bleeding. In our patient, the tumor had actually intussuscepted proximally into the third part of the duodenum causing near-total obstruction. This is the reason why it could be visualized on endoscopy. Based on these findings, we think that surgery was indicated in this patient. Although only a few large series comparing treatment modalities of GS are available in the literature, systemic chemotherapy seems to offer the most benefit.13 After reviewing 72 cases in the literature, Yamauchi and Yasuda concluded that the nonleukemic period after the diagnosis of GS was significantly longer in the patients who were treated with systemic chemotherapy, in comparison with the patients who did not receive chemotherapy.14 In conclusion, granulocytic sarcoma is a rare tumor which must be differentiated with intestinal lymphomas. Most patients with GS will progress to AML within several months, thus making the overall prognosis poor. However, AML-type chemotherapy appears to improve survival. Although surgical treatment of the tumor may not influence survival, it is indicated when tumor-related complications, such as intestinal obstruction or perforation, do occur.

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