Primary Non-Hodgkin’s Lymphoma of the Cervical Esophagus
Case Presentation and Discussion
Reynaldo O. Joson, MD, MHA, MHPEd, MS Surg
June 11, 1999
Abstract
A 62-year-old female with lymphoma of the cervical esophagus presented with dysphagia of solid foods. She had a goiter diagnosed one month prior to onset of dysphagia. A barium swallow showed no intrinsic esophageal lesion but with deviation.
She was operated with the diagnosis of thyroid carcinoma. Intraoperatively, a submucosal esophageal tumor was discovered. A cervical esophagectomy with tube pharyngostomy and tube esophagogastrostomy was done.
After the diagnosis of nonHodgkin’s lymphoma was known, a reconstruction of the alimentary canal using a left colon interposition was done less than 2 weeks after the first operation. Postoperative course was uneventful with respect to the reconstructive procedure. She, however, had hoarseness of voice and difficulty in swallowing, which were gradually improving at 3 weeks postop.
She is being considered for chemotherapy and/or radiotherapy.
Presentation and Discussion
A 62-year-old female was referred to me intraoperatively for opinion and management.
On data gathering from the referring surgeon, the following were gotten:
Neck mass was noted 6 months prior. Patient refused “thyroid operation” upon advised
by a surgeon.
Dysphagia was noted 5 months prior, characterized with difficulty in swallowing solid
foods. She could tolerate liquids.
Dysphagia was her main problem, more than her “goiter”.
A barium swallow was done which showed NO intrinsic lesion but with a deviation apparently from an extrinsic cause.
Patient was operated on with a preoperative diagnosis of thyroid tumor, probably
malignant.
Intraoperatively, the referring surgeon had completed a subtotal left lobectomy, isthmectomy, and total right lobectomy. He had difficulty establishing the definitive diagnosis. He was considering a right thyroid carcinoma encroaching on the esophagus based on the presence of “small” attachment of the right thyroid lobe to a mass on the esophagus. The esophagus had been opened at the area of the tumor.
When I scrubbed in, my first task was to make a diagnosis.
I evaluated the thyroid specimen that was previously removed by the referring surgeon. There was no dominant mass on the specimen. The thyroid specimen was nodular.
There was a 4 to 5-cm submucosal tumor in the cervical esophagus which was, I guessed, inadvertently opened by the referring surgeon during his dissection. There were also multiple nodes palpable in the superior mediastinum.
Initially, I thought of three differential diagnoses . Thyroid cancer with esophageal extension (because of the report of the referring surgeon that the thyroid was attached to the esophageal tumor);
esophageal carcinoma (based on prevalence; it is the most common esophageal cancer); and esophageal leiomyoma-leiomyosarcoma (because of the submucosal location of the tumor in the esophagus).
After repeatedly asking the referring surgeon on his findings (prior to my scrubbing in) and repeatedly inspecting and palpating the surgical field and the thyroid specimen, I told the team that thyroid carcinoma with esophageal extension was the least likely diagnosis among the three differential diagnoses. Bases: the thyroid specimen looked like a chronic thyroiditis and the external surface of the esophagus where the tumor was located was smooth, not rough, with no induration to suggest the presence of invasion from an adjacent organ such as the thyroid gland.
Weighing the probabilities of esophageal carcinoma and esophageal leiomyoma-sarcoma, the point against carcinoma was the submucosal location of the tumor while the point against a leiomyoma-sarcoma was the presence of multiple lymph nodes in the superior mediastinum. What favored carcinoma were the lymph nodes and what favored leiomyoma-sarcoma was the submucosal location of the tumor.
Based on prevalence, carcinoma is more common than leiomyoma-sarcoma. Since pattern recognition carries more weight than prevalence in the diagnostic process, I decided that the primary diagnosis was leiomyoma-sarcoma with carcinoma as secondary diagnosis. I placed the heaviest weight on the submucosal location of the tumor, thus, the primary diagnosis of leimyoma-sarcoma. In primarily considering leiomyoma-sarcoma, I was rationalizing that the lymph nodes might be nonspecific lymphadenopathy and not metastatic.
(Note: I never considered lymphoma during this time. It did not cross my mind. I have not seen one before. It is very rare.)
With an uncertain diagnosis, leiomyoma-sarcoma vs carcinoma, I asked myself: should I go for a frozen-section biopsy? (Note that the esophagus and the tumor were already open that a section can easily be done.) As a general rule, when one is not certain with the diagnosis, a frozen-section is indicated. However, there are other factors to consider before one finally decides to go for a frozen-section biopsy.
What I did was to consider first the management plan if the tumor were leiomyoma-sarcoma and carcinoma. If the management plans would NOT differ markedly then I don’t have to go for a frozen-section biopsy.
Whether the tumor is a leiomyoma-sarcoma or carcinoma, I had to resect at the very least the segment that contained the tumor, that is, doing a cervical esophagectomy. Repair and closure of the opened esophagus and tumor was out of the question. Attempting to do so would most certainly fail and result in a repair leak.
There was a point during my assessment of the proximal line of resection that I considered the need to do a total pharyngolaryngooesophagectomy (TPLO). The proximal line of resection might reach the hypopharynx, in which case, a TPLO might have to be done. If I decide to do a TPLO, whether the tumor is a leiomyoma-sarcoma or carcinoma, even if the treatment plans for both conditions are the same, I would certainly go for a frozen-section biopsy. The reason is that I need to be more definite on the diagnosis because of the mutilating and extensive nature of TPLO.
After further assessment of the possible level of the proximal line of resection, I decided I could have about 2-cm margin in the proximal line of resection. I could not tell with certainty the exact level of resultant proximal line of resection, which might be at the junction of the cervical esophagus and hypopharynx or even at the hypopharynx. Wherever it would be, I decided that TPLO should not be done at this time, if it could be avoided. If there is a need to do so, TPLO would be done in another time or day. The reason for this timing of TPLO operation was that the patient and relatives were not prepared for this radical operation. I too was not mentally and physically prepared for it.
With these considerations in mind, since my plan was just to do a cervical esophagectomy whatever the diagnosis might be, which for me was a toss-up between leiomyoma-sarcoma and carcinoma, I decided there was no need to do a frozen-section biopsy.
Thus, I did a cervical esophagectomy with about 2 cm proximal margin and about 3 cm distal margin with a resultant 10 cm gap between the proximal and distal ends of resection. I surmised my proximal margin was at the level of the junction of the hypopharynx and cervical esophagus because I was able to discern a short cuff of alimentary canal which I closed with simple interrupted silk sutures at the same time placing a polyethylene Fr. 14 tube, technically, a tube pharyngostomy to drain saliva. I closed the distal esophagus with a polyethylene Fr. 16 tube inserted up to the stomach, technically a tube esophagogastrostomy for enteral feeding purpose. A rubber drain was placed prior to the cervical incision closure.
I ended the operation with a plan to wait for the histopath result of the esophageal tumor and an excised lymph node before deciding on what to do next. Another surgery, chemotherapy, and radiation were the options kept open pending a definitive diagnosis.
The usual and necessary postoperative care measures, such as analgesics, fluid and nutritional support, antibiotics, and wound and tube care were given. Complications were also monitored. Except for hoarseness, which was most probably due to trauma to the recurrent laryngeal nerve caused by retraction as we tried to evaluate, expose, and evaluate the proximal esophagus, no other unwanted complications occurred.
We got the histopath result on the 4th day postop: NONHODGKIN’S LYMPHOMA of the esophagus. The node showed nonspecific lymphadenitis.
With the diagnosis of NonHodgekin’s lymphoma of the esophagus, there were 2 options I considered: to complete the treatment of the lymphoma first before reconstructing the alimentary canal or to reconstruct first. (Note: although the lymph node biopsy was negative for lymphoma, the multiple large lymph nodes that I felt at the superior mediastinum, I think, contained lymphoma. To complete the treatment, radiation and chemotherapy were considered.)
I placed more weight on the second option because the patient felt very depressed after learning that her problem of dysphagia worsened after the operation. She craved to eat. With this in mind, I requested for a CT scan of the chest to help us decide whether to reconstruct first or later after treatment with chemotherapy and/or radiation. If the CT scan would show heavy load of lymph nodes in the mediastinum, I will do the reconstruction after more treatment for the lymphoma. Since the CT scan showed minimal lymph node finding in the mediastinum, I decided to do the reconstruction first.
As to the timing of the reconstruction, I decided to do it as soon as possible, before fibrosis and contraction of the pharyngeal cuff set in.
After the first operation and before the second operation, I noticed the patient had no problem with aspiration. With this, I thought I don’t have to do a TPLO. Since the pathology was a lymphoma, as much as possible, I thought Ishould avoid a TPLO. I hope I can do a reconstruction using the pharyngeal cuff without unduly risking the patient to aspiration.
On the 8th or 9th day after the first operation, I did the reconstruction of the alimentary canal.
I decided to use a colonic interposition rather than a gastric pullup primarily because of the assured adequacy of length and lesser risk of aspiration. I decided to leave the thoracic esophagus because there was no indication to remove it. I tunneled the left colon based on a sigmoidal vessel into the retrosternal area. There were three anastomoses in the 2nd operation: colocolostomy, cologastrostomy, and pharyngocolostomy. A chest tube was placed on the right after I sensed I entered it during the retrosternal dissection.
What I found most difficult to anastomose was the pharyngocolostomy because of the limited field of exposure. Initially, I had difficulty finding a complete sturdy cylindrical pharyngeal cuff for a colon anastomosis. The silk sutures which I used in the previous operation helped me in locating the cuff. At the end of my single layer simple interrupted silk suture anastomosis, I had that feeling of “that’s the best that I can do for the situation.” I was anticipating a leak. I placed a rubber drain. Fortunately, no leak occurred.
The patient is now on its 3rd week post 2nd operation. She is hoarse. This was present even after the first operation. This is most likely due to trauma secondarily to retraction when we tried exposing the upper cervical esophagus and hypopharynx during the first and second operations. The right laryngeal nerve and the external branch of the superior laryngeal nerve were most likely traumatized. I am optimistic the voice will improve.
The patient is still having problem swallowing. Whenever she tries to eat, she would cough. This suggest she may be aspirating. This could be due to injury to the internal branch of the right superior laryngeal nerve, because this branch is sensory to the larynx above the glottis and to the regiion immediately around the entrance of the larynx. The difficulty in swallowing might also be due to incoordination of the musculatures such as the constrictors which are responsible for pharyngeal swallowing after the food is pushed to the oropharynx by the tongue.
Over the past 2 weeks, I received update from the referring surgeon that there was progressive improvement in swallowing characterized by more tolerability to food intake. She could now take in a little of “champurado.”
The plan from hereon is to give this patient chemotherapy once she is physically and mentally ready for the drugs. Estimated start of the chemotherapy is 6 to 8 weeks post 2nd operation.
The patient has been properly advised on the diagnosis and the treatment done so far as well as future treatment and follow-up plans.
She is very satisfied with the care given so far as shown by the gift of gratitude that she gave me after I did not charge her for my service (she is an aunt of the referring physician).
At this point, I can proudly say and with confidence that I have achieved my goals in the management of the patient. I have resolved her health problem, which was lymphoma of the esophagus causing dysphagia, in such a way that the disabilities and complications were acceptable, and in such a manner that the patient is satisfied.
Learning Issues
Lymphoma of the Esophagus
Lymphoma of the Gastrointestinal Tract
Epidemiology of lymphoma of the esophagus - global and local
Rare -
Of all lymphomas, less than 1%
Among all gastrointestinal lymphomas, least common after
stomach, small intestine, and large intestine (in decreasing order)
Marjority are secondary lymphoma of the esophagus
Primary lymphoma of the esophagus is rare
Majority are non-Hodgkin’s lymphoma
Some are Hodgkin’s lymphoma
Clinical presentation of lymphoma of the esophagus
Dysphagia secondary to an esophageal tumor
Gross pathology
Submucosal in location
Etiology
Not exactly known
Gold standard in diagnosis
Microscopic examination of tissue (biopsy)
Treatment of lymphoma of the esophagus
Same principles as in lymphoma of gastrointestinal tract
Surgery + chemotherapy + radiation
Prognosis:
5-year survival rate: 50-60%
Swallowing
Physiology of swallowing
Oral swallowing -----> Pharyngeal swallowing
five neuromuscular actions
velopharyngeal closure
pharyngeal peristalsis
laryngeal closure
laryngeal elevation and anterior movement
opening of cricopharyngeal region
Swallowing results after pharyngogastric reconstruction
Able to take solid diet - 60%
Able to take soft diet - 33%
Able to take liquid only - 4%
References:
Orvidas LJ, McCaffrey TV, Lewis JE, Kurtin PJ, Habermann TM. Lymphomas involving the esophagus.
Ann Otol Rhinol Laryngol 1994;103(11): 843-8.
Mayo Clinic
1945-1992 27 cases
3 - primary lymphomas
89% - non-Hodgekin’s lymphomas
41% - gastroesophageal junction
89% - with dysphagia
Dodd GD. Lymphoma of the hollow abdominal viscera. Radiol Clin North Am 1990;28(4):771-83.
M.D. Anderson
155 cases - 1. Stomach 2.Small intestine 3. Large intestine 4. Esophagus
Aozasa K, Tsujimoto M, Inoue A, et al. Primary gastrointestinal lymphoma. A clinicopathological study of 102 patients. Oncology 1985;42(2):97-103
Stomach, small intestine, large intestine, esophagus
Lam KH, Ho Cm, Wei WI, Wong J. Immediate reconstruction of pharyngeal defects - preference or reference. Arch Otolaryngol Head Neck Surg 1989; May: 608-612.
Logemann JA. Normal swallowing and the effects of oral cancer on normal deglutition. in Fee-Goepfert-Johns-Strong-Ward’s Head and Neck Cancer Vol 2 Toronto, B.C. Decker, Inc., 1990; 322 - 26.
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