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MEN2: Medullary thyroid cancer, pheochromocytoma

MEN2A: Parathyroid hyperplasia

MEN2B: Mucosal neuromas and marfanoid habitus

Treatment of MEN1

The primary concern is treating the hyperparathyroidism, which is more severe than in MEN2A. Surgical treatment with subtotal or total parathyroidectomy with arm reimplantation and cryo-preservation.

Resection of pancreatic islet cell neoplasm, if symptomatic

Treat pituitary tumors with bromocriptine, which is adequate in most cases.

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Treatment of MEN2

Total thyroidectomy for medullary thyroid cancer. Early operation for affected family members is done in early childhood before development of carcinoma.

Pheochromocytoma is treated with adrenalectomy after appropriate alpha blockade.

Hyperparathyroidism occurs in MEN2A, is milder than MEN1, and can be treated with subtotal parathyroidectomy.

Tumors of Endocrine Pancreas

Half are nonfunctional and half are functional (gastrinoma or insulinoma most common).

May be sporadic (occuring as solitary tumor) or with MEN1 (multifocal with tumors in pancreas and duodenal wall)

Insulinoma: 10% malignant; patients present with bizarre neurologic symptoms due to hypoglycemia; diagnose with high insulin in setting of hypoglycemia; imaging with CT and endoscopic ultrasound; treat by surgical enucleation; intraoperative ultrasound helpful to localize

Gastrinoma: 60% malignant; causes Zollinger -Ellison Syndrome; peptic ulcer disease and diarrhea common symptoms; confirm diagnosis with elevated gastrin; localize tumors with CT and endoscopic ultrasound; resect pancreatic tumors and excise duodenal tumors; most gastrinomas are located in the gastrinoma triangle, defined by the junction of the cystic and common bile ducts superiorly, the junction of the second and third portions of the duodenum inferiorly, and the junction of the neck and body of the pancreas medially; if unresectable, debulking will help symptoms (treat medically with H 2 blockers or PPI);

gastrectomy option to treat uncontrolled acid. P.354

Study Questions for Part V

Directions: Each of the numbered items in this section is followed by several possible answers. Select the ONE lettered answer that is BEST in each case.

1. A 40 -year-old man has a subtotal thyroidectomy performed for Graves' disease. Several hours later, he complains of difficulty breathing. On examination, he has stridor and a markedly swollen, tense neck wound. What should be one of the first steps in the management of this patient?

A Intubate with an endotracheal tube B Perform a tracheostomy

C Control the bleeding site in the operating room

D Open the wound to evacuate the hematoma

E Aspirate the hematoma

View Answer

2.A 50 -year-old hypertensive man has definitive biochemical evidence of a pheochromocytoma. Computed tomography (CT) scan and magnetic resonance imaging (MRI) do not reveal any abnormalities, and m- iodobenzylguanidine scanning is not readily available. What should be the next step in the management of this patient?

A Abdominal exploration

B Continued clinical observation C Mediastinoscopy

D Selective venous sampling E Mediastinal exploration View Answer

3.A 55 -year-old woman with progressive but episodic muscle weakness is diagnosed as having myasthenia gravis. Her chest radiograph is normal and reveals no evidence of mediastinal mass or tumor. What is the most definitive treatment that can be offered this patient?

A Prednisone B Neostigmine C Thymectomy

D Plasmapheresis E Atropine

View Answer

4.A first-degree relative of a patient found to have advanced medullary carcinoma of the thyroid gland is referred for further evaluation. Which screening measure is the choice for detection of medullary thyroid pathology?

A Careful physical examination B Serum calcitonin level

C Stimulated serum calcitonin level (calcium and pentagastrin) D Gastrin level

E Carcinoembryonic antigen (CEA) level View Answer

5.If a first-degree relative of a patient with MEN-2 A syndrome is found to have medullary pathology requiring surgical exploration of the thyroid gland, what should the preoperative screening include ?

A Serum cortisol level

B Fasting glucose and insulin C CT scan of the head

D Urinary aldosterone and renin

E Urinary vanillylmandelic acid and metanephrines View Answer

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6.A 60 -year-old female patient has a workup for episodic symptoms of palpitations, nervousness, and bizarre behavior, all of which tend to occur during fasting states. Biochemically, she is diagnosed as having an insulinoma. What is the best choice for localizing this tumor?

A CT scan B MRI

C Selective arteriography

D Percutaneous catheterization of the portal vein with selective venous sampling E Surgical exploration and intraoperative ultrasound

View Answer

7.A 55 -year-old female patient is evaluated for new onset of diabetes mellitus. Her medical history is largely unremarkable. Her physical examination is unrevealing except for the presence of an erythematous

skin rash. Her further evaluation should include an investigation of the possibility of which of the following?

AInsulinoma

BGlucagonoma

CGastrinoma

DCarcinoid tumor

EPancreatic cholera View Answer

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Answers and Explanations

1. The answer is D (Chapter 16, I D 2 e [4] [b] [ii]). Postoperative bleeding after thyroidectomy can cause airway compromise due to tracheal compression. The first step should be to open the wound to evacuate the hematoma, followed by a return to the operating room to control the bleeding site. Attempts to perform either endotracheal intubation or tracheostomy may be difficult until the external compression of the hematoma is relieved.

2. The answer is D (Chapter 16, II G 5 d). Although 90% of pheochromocytomas are located in the adrenal glands, they can occur in any tissue that is derived from neuroectoderm. When computed tomography (CT) scan and magnetic resonance imaging (MRI) do not identify a tumor, m-iodobenzylguanidine scanning can be helpful; however, this is not always available. Selective measurements of catecholamines drawn at various levels from the vena cava and its major branches should be obtained before surgical exploration.

3. The answer is C (Chapter 16, IV C 3 b). Myasthenia gravis is an autoimmune disease of neuromuscular transmission that causes skeletal muscle weakness. Parasympathomimetic drugs have been found to improve muscle strength in these patients. Prednisone has also been used with some success because of the autoimmune nature of this disease. Plasmapheresis may be effective in preparing the patient preoperatively. The treatment of choice for all forms of myasthenia, except purely ocular, appears to be thymectomy. An increased percentage of patients have permanent remission. The response to medication is improved in patients who do not achieve a complete remission.

4. The answer is C (Chapter 16, I F 5 c [2] [c]). All first -degree relatives of patients with medullary carcinoma of the thyroid gland should be screened for this disorder because it can occur in a familial pattern. Physical examination of the thyroid gland should be performed for the detection of any nodules. An increased serum calcitonin or an increased stimulated serum calcitonin test will also indicate underlying medullary pathology, either hyperplasia or carcinoma. The stimulated tests will detect disease at an earlier, more curable stage. Increased gastrin levels are associated with Zollinger -Ellison syndrome and are not part of this multiple endocrine adenomatosis (MEN) type 2 syndrome. Carcinoembryonic antigen (CEA) is elevated in some gastrointestinal malignancies.

5. The answer is E (Chapter 16, II G 4 a; Chapter 17, I B 2). Medullary carcinoma of the thyroid gland may present as a sporadic or familial form associated with MEN type 2A or 2B. Both are associated with pheochromocytomas. If a pheochromocytoma is present, it should be diagnosed and treated first to avoid the morbidity of cervical exploration in a patient with untreated pheochromocytoma. Urinary vanillylmandelic acid and metanephrines should be evaluated preoperatively.

6. The answer is E (Chapter 17, II B 4 a [2] ). The patient has had a definitive biochemical diagnosis of insulinoma. These tumors can be present anywhere in the pancreas. Because they are usually small in size, arteriography, CT, and MRI are less sensitive than they would be for larger tumors. With careful surgical exploration and intraoperative ultrasound, approximately 90% of these tumors can be localized at the time of surgery.

7. The answer is B (Chapter 17, II E 1). Glucagon -producing tumors of the pancreas secrete glucagon in

large amounts. Patients tend to present with new onset of diabetes mellitus (hyperglycemia). Affected individuals also characteristically have a migratory erythematous skin rash.

Chapter 18

Benign Lesions

Joseph R. Spiegel

Robert T. Sataloff

David A. Zwillenberg

I Introduction

Familiarity with the benign conditions reviewed in this chapter is essential to the physician who must distinguish life -threatening illnesses from those of little consequence, choose appropriate therapy, and avoid injudicious surgery.

A Overview

The most common neck mass is a reactive node, and these masses are most often secondary to bacterial or viral infections of the ear, nose, paranasal sinuses, teeth, tonsils, or skin and soft tissues of the head and neck.

Most neck masses in children are benign.

In adults, neck masses are more likely to be malignant.

The “rule of sevens” is a useful guide:

A mass that has been present for 7 days is inflammatory.

One present for 7 months is malignant.

One present for 7 years is congenital.

B Workup for acquired lesions

(see IV)

The history should be detailed, especially regarding:

Family history of malignancy

Past malignancy in the patient

Risk factors associated with malignancy, such as:

Smoking

Alcohol consumption

Exposure to radiation, certain fumes, sawdust, or other potential carcinogens

Recent relevant illnesses, such as:

Upper respiratory infection, sinusitis, or tonsillitis

Otitis or conjunctivitis

Dental problems

Physical examination should include careful inspection and palpation of the scalp, eyes, ears, nose, mouth (including the teeth and tonsils), hypopharynx, and nasopharynx for signs of infection, ulceration, or unsuspected abnormalities.

Laboratory tests may include:

Complete blood count and differential

Chest radiograph

Tuberculin test for tuberculosis

A heterophil titer (monospot test) for mononucleosis

Thyroid function tests or thyroid scan

Serologic tests for syphilis

Viral titers, especially for Epstein -Barr virus, which is associated with nasopharyngeal carcinoma and Burkitt's lymphoma

Radiologic studies may include soft tissue radiographs of the neck, xeroradiograms, a barium swallow, and a complete gastrointestinal series or scanning procedures such as computed

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tomography (CT), magnetic resonance imaging (MRI), bone scan, or other radioisotope scans.

FIGURE 18-1 Algorithm for evaluation and treatment of a neck mass. FNA, fine-needle aspiration; PE, physical examination.

Endoscopy is indicated to search for the tumor if a primary neoplasm is suspected. Endoscopic biopsy and radiologic studies should precede any incision in the neck (see Chapter 19, II B )

Treatment depends on the findings during the workup (Fig. 18 -1).

Antibiotics should be administered if a bacterial infection is suspected.

Antituberculous drugs may be needed.

Consultation with a specialist in another field may be helpful.

A dental consultation may be useful if the teeth seem to be the source of a problem.

If dandruff, scabies, or another dermatologic condition is noted, a dermatology consultation is indicated.

If a mass does not shrink significantly or disappear within a reasonable time (usually 6 weeks), then surgical treatment or biopsy may be indicated.

If cervical adenopathy persists, then:

The presence of enlarged or cryptic tonsils is believed by many to be an indication for tonsillectomy.

Equivocal or abnormal dental findings are an indication for dental treatment.

If a source of infection is not found, then persistent cervical adenopathy is an indication for excisional biopsy after a complete evaluation for malignancy.

A neck mass biopsy is the last step in a proper workup. Fine -needle aspiration can be used to diagnose carcinoma, but it is usually inadequate to define lymphoma.

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TABLE 18-1 Neck Abscesses

Signs and Symptoms

Pain

Swelling

Dysphagia

Dyspnea

Leukocytosis

Fever

Air in soft-tissue radiograph

Treatment

Airway protection

Incision and drainage

Antibiotics

II Neck Abscesses

A Overview

A patient presenting with fever and a painful, fluctuant neck mass most probably has an abscess (Table 18 -1).

The source of infection should be identified, and drainage should be carried out.

Owing to the danger to the carotid artery, airway, and cranial nerves, deep neck abscesses should be treated only by those knowledgeable in the standard techniques and anatomy of the area. They should be treated on an emergency basis.

B Types of abscesses

Bezold's abscesses are neck abscesses that arise from infection in the middle ear or mastoid.

Ludwig's angina is an abscess that occupies the sublingual space.

It generally arises from a dental source.

It can cause death from airway obstruction and, therefore, frequently requires tracheostomy.

Parapharyngeal space abscesses arise from the posterior teeth or tonsils and can affect the carotid sheath structures and multiple cranial nerves. They can cause mediastinitis and carotid “blowout” (i.e., erosion of the artery wall leading to massive hemorrhage).

Retropharyngeal abscesses arise from infected retropharyngeal nodes or extension from other spaces. They can lead to airway obstruction or mediastinitis.

Peritonsillar abscesses (quinsy) arise as a complication of acute tonsillitis.

They present with ipsilateral palatal edema, contralateral deviation of the uvula, “hot potato” voice, trismus, and dysphagia. The patient may have only a low -grade fever or be afebrile.

They are the most common abscesses in the parapharyngeal space.

III Congenital Masses (Table 18-2)

A Parenchymal cysts

Thymic cysts

Embryology. The thymus arises from the third pharyngeal pouch and migrates caudally and medially to descend into the superior mediastinum.

During this descent, an attachment may remain in the neck.

Thymic tissues may present in the neck as separate nodules of mature thymus or may occur in association with ciliated or columnar epithelial remnants of the pharyngeal outpouching.

Thymic cysts may occur anywhere on a line from the mandibular angle to the suprasternal notch.

Characteristics