Saccular Mucocele and Donald F. N.
\s=b\ Saccular mucoceles are a definite
remains alive and well four years after excision of a T4 carcinoma, although a unilateral radical neck dissection was performed two years ago.
(Arch Otolaryngol 103:232-234, 1977)
nal
it is remembered that exter-
laryngoceles
were
recog-
nized as a clinical entity early in the 19th century and that this condition has excited some sporadic interest ever since, it is surprising that confusion regarding both incidence and cause still persists. By common consent, laryngoceles are divided into external, internal, and combined, though whether a pure internal laryngocele is ever clinically diagnosed is doubtful, since by definition they must only contain air and thus produce minimal symptoms. The supposition that this is trapped within an expanded saccule is obviously unlikely since it would be readily absorbed, and in any case, the "valve" that is found at the mouth of the saccule, which is described by Delahunty and Cherry,1
Accepted
for publication Nov 30, 1976. the Institute of Laryngology Otology, London. Reprints not available. From
Cancer
Harrison, MD, MS, FRCS
entity and probably occur more frequently than is supposed. Confusion with internal laryngoceles is possibly due to lack of understanding of basic anatomy and physiology. A 49-year-old man had a saccular mucocele that was possibly associated with laryngeal cancer, although a definitive diagnosis was only made two years later. The laryngectomy specimen was serially sectioned and showed an internal laryngocele on the side that was not operated on. This patient
When
Laryngeal
and
is clearly designed only to retain and then to direct saccular mucus. My own studies on 150 coronally sectioned human larynxes has confirmed their findings. A study of the relevant literature emphasizes the confusion of thought that exists, which is primarily based on inadequate experience and fundamental knowledge. Stell and Maran2 have exploded the myth that repeated increase in intralaryngeal pressure is the primary cause of laryngocele formation. It is now clear that anatomic variations in the size of the saccule are sufficient to explain the occasional expansion of this structure under appropriate physiologic conditions. Consequently, one supposes that all external laryngoceles also must have an internal component. Undoubtedly, if one adopts this more realistic diagnostic criteria, all types of laryngoceles are rare in man, although air sacs of one kind or another are common in most species of Mammalia. These are asymptomatic and are essentially functional, and my own continuing studies on the serially sectioned mammalian larynx shows that many are related to the laryngeal ventricles, having been previously undetected by the comparative zoologist (Fig 1). An association between laryngocele and laryngeal cancer has been reported by many authors, such as Pietrantoni et al.3 Their estimate of just over 6% of patients with laryngeal cancer and a laryngocele was based on radiologic diagnosis supported by an unsophisticated evaluation of 31 excised larynxes divided into several sections. How these estimations were made is obscure, and the availability of large numbers of serial-
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
ly sectioned whole larynxes, which permits a precise evaluation of this problem, has failed to substantiate their findings. In fact, there is not the slightest reason to expect such an association, since although an extensive saccule may provide ready access for a malignant tumor to pass from supraglottis to preepiglottic space, blockage of the mouth of the saccule is more likely to trap mucus than air.
The studies of Hast4 and others have now established that the laryngeal ventricle develops as an outpouching of the laryngeal cavity around the second month (crown rump 22 to 24 mm). The apex of this evagination forms the saccule, which consequently lies lateral to the false cord. Serial coronal sections have shown the variation in size of this pouch, complexity of mucosal lining, and richness of the mucous glands. It would not be surprising, therefore, if a malignant tumor, instead of spreading submucosally along the saccule, blocked the mouth. This might happen primarily or as a result of radiotherapy or limited surgical excision. Although reports of mucocele formation in the saccule are uncommon, this is a clearly recognized clinical entity, and I believe that it is commoner than any other form of laryngocele. A history of hoarseness associated with a swelling of the false cord and radiologic appearances similar to those shown in Fig 2 is virtually diagnostic. Occasionally the patient's symptoms are relieved for a short period by the coughing up of a clear fluid\p=m-\possiblya temporary decompression of the mucocele (sometimes called an encysted laryngocele). Excision of the distended saccule via a
Fig 2 Tomogram of larynx showing an air containing swelling expanding left false cord but apparently communicating with laryngeal cavity. At operation this con¬ —
Fig 1.—Larynx and external air sacs of adult male plains Vizcacha (Lagostomus maximus). This South American burrowing rodent is known for variety of its vocalization but no swelling of neck has ever been noted in captivity or wild.
3.—Serial section 1230 showing mouth of right saccule and carcinoma in relation to medial surface (hematoxylin-eosin, orig¬ inal magnification x11).
Fig
tained mucus, not air, and mouth of saccule was blocked.
Fig 4.—Serial section 1120 showing erosion of both thyroid cartilages, tumor growing into strap muscles on both sides, and small internal
magnification
laryngocele on 1.5).
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
left side
(hematoxylin-eosin, original
small window in the thyroid cartilage in six patients has verified this diag¬ nosis and shows the sac to be lined with ciliated columnar epithelia and filled with clear mucus, except in one patient where a pyocele had developed due to infection. The cause of these mucoceles remained indeterminate (as in many frontal sinus mucoceles), and the surprise is that the condition does not occur more frequently. Per¬ haps it does, but when moderate in size may cause little symptoms or remains undiagnosed. In 1972, Cassady and Mathur3 reported a patient who developed a saccular mucocele after treatment for laryngeal cancer. Although this pa¬ tient had undergone a left cordectomy five years before, the mucocele was related to local recurrence of his laryn¬ geal neoplasm. The patient reported by Lund" is rather more of a diag¬ nostic disaster, since four months after removing a large internal laryn¬ gocele the patient was found to have a carcinoma affecting both vocal cords. To add to the confusion, examination of the operative specimen showed a right external laryngocele. In this patient, one must assume the coinci¬ dental occurrence of a laryngeal cancer and bilateral congenital laryn¬
goceles. The patient whose case is reported in this paper has provided a unique opportunity for evaluating some of these perplexing problems, since he has been under my care for six years and his operative specimen has been processed according to the routine whole organ serially sectioning pro¬ gram, which has been standard proce¬ dure for over eight years in my unit.7 REPORT OF A CASE
A 49-year-old man initially was seen in February 1970 with a history of gradually increasing hoarseness for about five years. He underwent laryngoseopy on several
occasions elsewhere and received electroconvulsive therapy for a supposed psycho¬ somatic condition related to his "supposed" hoarseness. Results of a direct laryngos¬ eopy showed a smooth swelling of the right false cord but no obvious abnormality else¬ where in the larynx, particularly in the ventricle on that side. Tomograms showed the features illustrated in Fig 2, and a
provisional diagnosis of saccular mucocele made. Early in March 1970, this was removed via a laryngofissure approach, a technique no longer used by me for these lesions, and a mucous containing a cyst was was
dissected from beneath the false cord mucous membrane. This cyst extended up to the thyrohyoid membrane but at its junction with the ventricle was closely related to a small friable granulation. The latter was removed for histologie examina¬ tion and was reported as a squamous carci¬ noma. The patient was referred for a cura¬ tive dosage of radiotherapy, and mean¬ while this biopsy material was sent to the National Ear, Nose, and Throat Tumour Panel. Three days after commencement of radiotherapy, they reported that this was not a carcinoma. Not unexpectedly, the patient was somewhat upset by this varia¬ tion in opinion, but with the knowledge that it was preferable not to have a cancer, he returned home and came for routine
follow-up. He remained hoarse with reduced exer¬ cise tolerance and edematous false cords. In June 1972 he was readmitted for an exploratory laryngofissure to see if an improvement in airway could be produced. At operation both false cords appeared to be replaced by dense fibrotic tissue, and the mucosa on the upper surface of the left vocal cord appeared macroscopically abnor¬ mal. Biopsy specimens were taken from all these areas and showed extensive invasion by well-differentiated squamous carcino¬ ma with evidence to suggest an origin from respiratory-type epithelium. A few days later, the larynx was removed together with a portion of skin containing the two previous laryngofissure incisions. Despite the emotional shock relating to this unexpected diagnosis, the patient rehabilitated well until March 1974, when he developed a firm mass in a lower, deep cervical lymph node on the left side of his neck. This was removed via a radical neck dissection, and he has remained well (he was last seen in April 1976, just over six years after his first surgical procedure). Because of pressure on the processing staff, and the fact that we still believe the best sections are obtained by the use of the double-embedding technique in celloidin," the sections of this larynx only became available this year. However, this has given us an ample opportunity for adequate follow-up; it is four years since total laryngectomy. The neck dissection specimen was processed according to the method advocated by McKelvie" and contained 30 lymph nodes, of which only one contained metastatic carcinoma. This was 5 cm and adjacent to a thrombosed blood vessel that it was invading. Figure 3 is a high-power
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
view of the mouth of the right saccule and shows this to be surrounded by carcinoma, while Fig 4 (section 1120 of the whole series) shows clearly that both thyroid cartilages have been eroded and that disease is well outside the larynx and in¬ volving the strap muscles. In addition, there is an obvious, and unexpected, dila¬ tion of the end of the left saccule. This reaches the thyrohyoid membrane, and since it is in free communication with the laryngeal airway, it might well be termed an internal laryngocele. The rugose nature of the saccule is also obvious and provides a clear picture of the extensive nature of this mucus-producing surface.
COMMENT
Further review of the February 1970 biopsy specimen has not sub¬ stantiated a diagnosis of carcinoma, and the question as to whether disease existed at that time remains un¬ proved. In view of the findings shown in Fig 4, it is possible, of course, that bilateral, dilated saccules existed at that time and the patient undoubtedly had a right saccular mucocele. Wheth¬ er this was secondary to neoplastic obstruction of the saccular mouth will never be known, but he must have possessed considerable immunologie competence to have survived for years with a T, laryngeal cancer. It was possible, then, to have a delay of over two years between the first laryngo¬ fissure and a definitive diagnosis of neoplasia. The patient has never smoked, and despite the vagarities of both diagnosis and treatment, he remains grateful to us but incensed with those who recommended his electroconvulsive therapy. References 1. Delahunty JE, Cherry J: The laryngeal saccule. J Laryngol Otol 83:803-815, 1969. 2. Stell PM, Maran AGD: Laryngocele. J Laryngol Otol 89:915-924, 1975. 3. Pietrantoni L, Felisati D, Finzi A: Laryngocele and laryngeal cancer. Ann Otol Rhinol
Laryngol 68:100-107, 1959. 4. Hast M: Developmental anatomy of the larynx. Otolaryngol Clin North Am 3:413-438, 1970. 5. Cassady
larynx
CL, Mathur AK: Mucocele of the
sequela
of carcinoma. Arch Otolar1972. 6. Lund WS: A case of external and internal laryngocele associated with carcinoma of the larynx. J Laryngol Otol 74:260-265, 1960. 7. Harrison DFN: Pathology and management of subglottic cancer. Ann Otol Rhinol Laryngol as a
yngol 98:60-62,
80:1-7, 1971.
8. McKelvie P: Metastatic routes in the neck. Can J Otol 3:473-479, 1974.
\s=b\ Saccular mucoceles are a definite
remains alive and well four years after excision of a T4 carcinoma, although a unilateral radical neck dissection was performed two years ago.
(Arch Otolaryngol 103:232-234, 1977)
nal
it is remembered that exter-
laryngoceles
were
recog-
nized as a clinical entity early in the 19th century and that this condition has excited some sporadic interest ever since, it is surprising that confusion regarding both incidence and cause still persists. By common consent, laryngoceles are divided into external, internal, and combined, though whether a pure internal laryngocele is ever clinically diagnosed is doubtful, since by definition they must only contain air and thus produce minimal symptoms. The supposition that this is trapped within an expanded saccule is obviously unlikely since it would be readily absorbed, and in any case, the "valve" that is found at the mouth of the saccule, which is described by Delahunty and Cherry,1
Accepted
for publication Nov 30, 1976. the Institute of Laryngology Otology, London. Reprints not available. From
Cancer
Harrison, MD, MS, FRCS
entity and probably occur more frequently than is supposed. Confusion with internal laryngoceles is possibly due to lack of understanding of basic anatomy and physiology. A 49-year-old man had a saccular mucocele that was possibly associated with laryngeal cancer, although a definitive diagnosis was only made two years later. The laryngectomy specimen was serially sectioned and showed an internal laryngocele on the side that was not operated on. This patient
When
Laryngeal
and
is clearly designed only to retain and then to direct saccular mucus. My own studies on 150 coronally sectioned human larynxes has confirmed their findings. A study of the relevant literature emphasizes the confusion of thought that exists, which is primarily based on inadequate experience and fundamental knowledge. Stell and Maran2 have exploded the myth that repeated increase in intralaryngeal pressure is the primary cause of laryngocele formation. It is now clear that anatomic variations in the size of the saccule are sufficient to explain the occasional expansion of this structure under appropriate physiologic conditions. Consequently, one supposes that all external laryngoceles also must have an internal component. Undoubtedly, if one adopts this more realistic diagnostic criteria, all types of laryngoceles are rare in man, although air sacs of one kind or another are common in most species of Mammalia. These are asymptomatic and are essentially functional, and my own continuing studies on the serially sectioned mammalian larynx shows that many are related to the laryngeal ventricles, having been previously undetected by the comparative zoologist (Fig 1). An association between laryngocele and laryngeal cancer has been reported by many authors, such as Pietrantoni et al.3 Their estimate of just over 6% of patients with laryngeal cancer and a laryngocele was based on radiologic diagnosis supported by an unsophisticated evaluation of 31 excised larynxes divided into several sections. How these estimations were made is obscure, and the availability of large numbers of serial-
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
ly sectioned whole larynxes, which permits a precise evaluation of this problem, has failed to substantiate their findings. In fact, there is not the slightest reason to expect such an association, since although an extensive saccule may provide ready access for a malignant tumor to pass from supraglottis to preepiglottic space, blockage of the mouth of the saccule is more likely to trap mucus than air.
The studies of Hast4 and others have now established that the laryngeal ventricle develops as an outpouching of the laryngeal cavity around the second month (crown rump 22 to 24 mm). The apex of this evagination forms the saccule, which consequently lies lateral to the false cord. Serial coronal sections have shown the variation in size of this pouch, complexity of mucosal lining, and richness of the mucous glands. It would not be surprising, therefore, if a malignant tumor, instead of spreading submucosally along the saccule, blocked the mouth. This might happen primarily or as a result of radiotherapy or limited surgical excision. Although reports of mucocele formation in the saccule are uncommon, this is a clearly recognized clinical entity, and I believe that it is commoner than any other form of laryngocele. A history of hoarseness associated with a swelling of the false cord and radiologic appearances similar to those shown in Fig 2 is virtually diagnostic. Occasionally the patient's symptoms are relieved for a short period by the coughing up of a clear fluid\p=m-\possiblya temporary decompression of the mucocele (sometimes called an encysted laryngocele). Excision of the distended saccule via a
Fig 2 Tomogram of larynx showing an air containing swelling expanding left false cord but apparently communicating with laryngeal cavity. At operation this con¬ —
Fig 1.—Larynx and external air sacs of adult male plains Vizcacha (Lagostomus maximus). This South American burrowing rodent is known for variety of its vocalization but no swelling of neck has ever been noted in captivity or wild.
3.—Serial section 1230 showing mouth of right saccule and carcinoma in relation to medial surface (hematoxylin-eosin, orig¬ inal magnification x11).
Fig
tained mucus, not air, and mouth of saccule was blocked.
Fig 4.—Serial section 1120 showing erosion of both thyroid cartilages, tumor growing into strap muscles on both sides, and small internal
magnification
laryngocele on 1.5).
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
left side
(hematoxylin-eosin, original
small window in the thyroid cartilage in six patients has verified this diag¬ nosis and shows the sac to be lined with ciliated columnar epithelia and filled with clear mucus, except in one patient where a pyocele had developed due to infection. The cause of these mucoceles remained indeterminate (as in many frontal sinus mucoceles), and the surprise is that the condition does not occur more frequently. Per¬ haps it does, but when moderate in size may cause little symptoms or remains undiagnosed. In 1972, Cassady and Mathur3 reported a patient who developed a saccular mucocele after treatment for laryngeal cancer. Although this pa¬ tient had undergone a left cordectomy five years before, the mucocele was related to local recurrence of his laryn¬ geal neoplasm. The patient reported by Lund" is rather more of a diag¬ nostic disaster, since four months after removing a large internal laryn¬ gocele the patient was found to have a carcinoma affecting both vocal cords. To add to the confusion, examination of the operative specimen showed a right external laryngocele. In this patient, one must assume the coinci¬ dental occurrence of a laryngeal cancer and bilateral congenital laryn¬
goceles. The patient whose case is reported in this paper has provided a unique opportunity for evaluating some of these perplexing problems, since he has been under my care for six years and his operative specimen has been processed according to the routine whole organ serially sectioning pro¬ gram, which has been standard proce¬ dure for over eight years in my unit.7 REPORT OF A CASE
A 49-year-old man initially was seen in February 1970 with a history of gradually increasing hoarseness for about five years. He underwent laryngoseopy on several
occasions elsewhere and received electroconvulsive therapy for a supposed psycho¬ somatic condition related to his "supposed" hoarseness. Results of a direct laryngos¬ eopy showed a smooth swelling of the right false cord but no obvious abnormality else¬ where in the larynx, particularly in the ventricle on that side. Tomograms showed the features illustrated in Fig 2, and a
provisional diagnosis of saccular mucocele made. Early in March 1970, this was removed via a laryngofissure approach, a technique no longer used by me for these lesions, and a mucous containing a cyst was was
dissected from beneath the false cord mucous membrane. This cyst extended up to the thyrohyoid membrane but at its junction with the ventricle was closely related to a small friable granulation. The latter was removed for histologie examina¬ tion and was reported as a squamous carci¬ noma. The patient was referred for a cura¬ tive dosage of radiotherapy, and mean¬ while this biopsy material was sent to the National Ear, Nose, and Throat Tumour Panel. Three days after commencement of radiotherapy, they reported that this was not a carcinoma. Not unexpectedly, the patient was somewhat upset by this varia¬ tion in opinion, but with the knowledge that it was preferable not to have a cancer, he returned home and came for routine
follow-up. He remained hoarse with reduced exer¬ cise tolerance and edematous false cords. In June 1972 he was readmitted for an exploratory laryngofissure to see if an improvement in airway could be produced. At operation both false cords appeared to be replaced by dense fibrotic tissue, and the mucosa on the upper surface of the left vocal cord appeared macroscopically abnor¬ mal. Biopsy specimens were taken from all these areas and showed extensive invasion by well-differentiated squamous carcino¬ ma with evidence to suggest an origin from respiratory-type epithelium. A few days later, the larynx was removed together with a portion of skin containing the two previous laryngofissure incisions. Despite the emotional shock relating to this unexpected diagnosis, the patient rehabilitated well until March 1974, when he developed a firm mass in a lower, deep cervical lymph node on the left side of his neck. This was removed via a radical neck dissection, and he has remained well (he was last seen in April 1976, just over six years after his first surgical procedure). Because of pressure on the processing staff, and the fact that we still believe the best sections are obtained by the use of the double-embedding technique in celloidin," the sections of this larynx only became available this year. However, this has given us an ample opportunity for adequate follow-up; it is four years since total laryngectomy. The neck dissection specimen was processed according to the method advocated by McKelvie" and contained 30 lymph nodes, of which only one contained metastatic carcinoma. This was 5 cm and adjacent to a thrombosed blood vessel that it was invading. Figure 3 is a high-power
Downloaded From: http://archotol.jamanetwork.com/ by a Monash University Library User on 06/21/2015
view of the mouth of the right saccule and shows this to be surrounded by carcinoma, while Fig 4 (section 1120 of the whole series) shows clearly that both thyroid cartilages have been eroded and that disease is well outside the larynx and in¬ volving the strap muscles. In addition, there is an obvious, and unexpected, dila¬ tion of the end of the left saccule. This reaches the thyrohyoid membrane, and since it is in free communication with the laryngeal airway, it might well be termed an internal laryngocele. The rugose nature of the saccule is also obvious and provides a clear picture of the extensive nature of this mucus-producing surface.
COMMENT
Further review of the February 1970 biopsy specimen has not sub¬ stantiated a diagnosis of carcinoma, and the question as to whether disease existed at that time remains un¬ proved. In view of the findings shown in Fig 4, it is possible, of course, that bilateral, dilated saccules existed at that time and the patient undoubtedly had a right saccular mucocele. Wheth¬ er this was secondary to neoplastic obstruction of the saccular mouth will never be known, but he must have possessed considerable immunologie competence to have survived for years with a T, laryngeal cancer. It was possible, then, to have a delay of over two years between the first laryngo¬ fissure and a definitive diagnosis of neoplasia. The patient has never smoked, and despite the vagarities of both diagnosis and treatment, he remains grateful to us but incensed with those who recommended his electroconvulsive therapy. References 1. Delahunty JE, Cherry J: The laryngeal saccule. J Laryngol Otol 83:803-815, 1969. 2. Stell PM, Maran AGD: Laryngocele. J Laryngol Otol 89:915-924, 1975. 3. Pietrantoni L, Felisati D, Finzi A: Laryngocele and laryngeal cancer. Ann Otol Rhinol
Laryngol 68:100-107, 1959. 4. Hast M: Developmental anatomy of the larynx. Otolaryngol Clin North Am 3:413-438, 1970. 5. Cassady
larynx
CL, Mathur AK: Mucocele of the
sequela
of carcinoma. Arch Otolar1972. 6. Lund WS: A case of external and internal laryngocele associated with carcinoma of the larynx. J Laryngol Otol 74:260-265, 1960. 7. Harrison DFN: Pathology and management of subglottic cancer. Ann Otol Rhinol Laryngol as a
yngol 98:60-62,
80:1-7, 1971.
8. McKelvie P: Metastatic routes in the neck. Can J Otol 3:473-479, 1974.
