A Collection of Cancerous Conditions
Basal Cell Carcinoma of the External Ear
William F. Keenan Jr, MD
Williamsport Regional Medical Center, Williamsport, Pennsylvania
Basal cell carcinomas (BCCs) are the most common cancer in the United States. They most often occur in sun-exposed areas.1 Because the ears are often exposed to the sun’s radiation, the pinnae are a common site for the development of BCCs. Treating BCCs of the external ear poses a challenge, in part because the skin is firmly attached to the underlying cartilage, and in part because the cartilage itself can be easily damaged.
Patient A was a 75-year-old woman who felt a slowly growing lump inside the upper left pinna. Her medical history was remarkable only for hypertension. As a child, she often had been outdoors; individuals in her generation seldom used sunscreens, since there was little appreciation for their benefits. She presented with a nodular lesion with a central depression (Figure 1), consistent with a nodular BCC, the diagnosis of which was ultimately proven by biopsy results. The depression likely represented an incipient ulcer, given the fact that these tumors outgrow the blood supply from their abnormal vessels.
We discussed treatment options with the patient. Simple excision would have been difficult because of the lesion’s location, and Mohs micrographic surgery also would have been difficult in this location; either technique likely would have required a skin graft. Curettage and desiccation would have left a large scar and carried the risk of cartilage damage. We recommended, and she opted for, a course of radiation therapy.
BCCs are radiation-sensitive, and treatment usually has good results.2 Because the underlying cartilage is also radiation-sensitive, small repeated treatments are required to avoid damage to the cartilage. This woman underwent 19 treatments and had minimal short-term complications, with excellent cosmesis. Figure 2 shows the appearance 1 year after the diagnosis, with no evidence of tumor recurrence.
Patient B was a 65-year-old man who had developed a scab on his right ear that had slowly enlarged. His history was positive for multiple epiphyseal dysplasia. He, too, had seldom used sunscreens as a child but had often been outdoors. He was an avid fisherman and almost never wore a hat while fishing.
On physical examination, an ulcerated lesion was present under the scab (Figure 3); the lesion proved to be an infiltrative type of BCC on biopsy.
We discussed the treatment options with him. Because of the more aggressive nature of infiltrating BCCs, simple excision would have been difficult. He chose radiation therapy and underwent 20 brief treatments. The radiation therapy resulted in an excellent cosmesis, with no tumor regrowth at 1 year’s follow-up (Figure 4).
Radiation therapy can be used to treat BCCs and squamous cell carcinomas. Depending on the location (eg, the external ear), multiple treatments may be needed to avoid damage to underlying structures. Lower-extremity lesions may result in poorly healing ulcers. Because the radiation can cause chromosomal damage, the potential exists for additional cancers to arise years later, but this is less of a concern with most elderly patients given the relatively long lag time between treatment and the appearance of new cancers.
- Epidermal nevi, neoplasms, and cysts. In: James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 12th ed. Philadelphia, PA: Elsevier; 2016:chap 29.
- McGregor S, Minni J, Herold D. Superficial radiation therapy for the treatment of nonmelanoma skin cancers. J Clin Aesthet Dermatol. 2015;8(12):12-14.
Dedifferentiated Liposarcoma of the Hypopharynx
Walter A. Schroeder Jr., DO, MD; Mark W. Hosler, MD; and Lee Beckwith, MD
Southeast Health, Cape Girardeau, Missouri
A 61-year-old man presented with a 2-month history of vague changes in his voice and increasing dysphagia. He denied shortness of breath, hoarseness, pain, or bleeding. The patient’s medical history was remarkable for hypertension. He did not use tobacco or abuse alcohol.
A flexible fiberoptic examination revealed a well-encapsulated yellow mass with no ulceration obscuring the visualization of the posterior vocal folds. The true vocal folds were mobile. Saliva pooled in the supraglottic larynx but cleared with swallowing. There was no cervical adenopathy.
The patient agreed to undergo direct laryngoscopy preceded by imaging prior to surgery. Computed tomography (CT) scanning with contrast of the tissue of the neck demonstrated a 2.9 × 2.6 × 2.5-cm, well-circumscribed, mildly heterogeneous mass (Figure 1). Severe narrowing of the supraglottic airway was noted, but with no evidence of invasion or erosion into soft tissue or osseous structures. No adenopathy was noted.
Figure 1: CT scans with contrast of the tissue of the neck. Coronal view (1A) showed a mass positioned superior to the arytenoids. Sagittal view (1B) demonstrated an encapsulated mass inferior to epiglottis extending to the posterior pharynx. The mass mea- sured 2.9×2.6×2.5 cm and was well circumscribed and mild- ly heterogeneous. Severe narrowing of the supraglottic airway was noted, but with no evidence of invasion or erosion into soft tissue or osseous structures. No adenopathy was noted.
The tumor was resected along its capsule using a transoral approach under general anesthesia. The tumor was loosely attached to the posterior pharyngeal wall. Gross pathology revealed a well-defined, smooth, yellow encapsulated mass (Figure 2). Histopathology test results identified the mass as a dedifferentiated high-grade liposarcoma characterized by atypical spindle cells and mitoses (Figure 3).
Figure 2: Gross pathology demonstrated a well-encapsulated mass.
Figure 3: Histological specimen showing a lipoblast (3A) and a dedifferentiated area with atypical spindle cells and mitoses (3B) (hematoxylin-eosin, original magni cation ×400).
The patient was free of symptoms following surgery. He underwent postoperative radiation therapy.
Discussion. Liposarcoma of the hypopharyngeal region is rare. The most common symptoms are dysphagia, change in voice, difficulty clearing secretions, mild discomfort, and weight loss.1 The duration of symptoms ranges from several weeks to several years.2 The symptoms depend on the size and location of the tumor. Fiberoptic endoscopy can be used to evaluate the size and location of the tumor for treatment planning. Preoperative imaging provides additional information to assist in determining the surgical approach.
The 4 histological subtypes of liposarcoma are well-differentiated, dedifferentiated, myxoid/round cell, and pleomorphic.3 The tumors most commonly occur in adult men. Most of the tumors are well-differentiated liposarcomas.4 Liposarcoma often has a gross appearance of a benign lipoma, but CT imaging results demonstrating a heterogeneous mass are suggestive of a liposarcoma.3
The treatment of these tumors, regardless of histological grade, is complete surgical excision.1-5 Incomplete excision can result in local recurrence. Cervical metastases are rare, and lymph node resection is usually not recommended. Postoperative radiotherapy should be considered in patients with high-grade tumors, positive margins, large tumors, local extension, or complex anatomical subsites.5
The condition can be misdiagnosed because of its benign appearance. Liposarcomas of the head and neck were initially misdiagnosed in 33% of patients in one retrospective case series.5 Patients with recurrent lipomas may benefit from review of the original tumor.
A transoral approach can be used to remove the tumor, provided the entire tumor can be visualized. In our patient’s case, a combination of a rigid laryngoscope and mouth gag was used to visualize the tumor. Postoperative radiation should be considered in select cases. Continued surveillance of patients with liposarcoma of the hypopharynx is prudent.
- Reed JM, Vick EG. Hypopharyngeal liposarcoma. Otolaryngol Head Neck Surg. 1996;114(3):499-500.
- Wenig BM, Heffner DK. Liposarcomas of the larynx and hypopharynx: a clinicopathologic study of the eight new cases and a review of the literature. Laryngoscope. 1995;105(7 pt 1):747-756.
- Yoshii R, Yokoyama J, Ohba S, et al. Impact of endoscopic surgery for an uncommon liposarcoma of the posterior wall of the oropharynx: a case report. Head Neck Oncol. 2012;4(2):63.
- Kodiyan J, Rudman JR, Rosow DE, Thomas GR. Lipoma and liposarcoma of the larynx: case reports and literature review. Am J Otolaryngol. 2015;36(4):611-615.
- Davis EC, Ballo MT, Luna MA, et al. Liposarcoma of the head and neck: the University of Texas M. D. Anderson Cancer Center experience. Head Neck. 2009;31(1):28-36.
Jessica G. Gelman, MD, MPH; Kimberly Berger, MD, MPH; and Daniel Lichtstein, MD
University of Miami Miller School of Medicine, Miami, Florida
A 65-year-old woman presented with a 1-year history of progressively worsening shortness of breath, fatigue, and a 40.8-kg unintentional weight loss. She reported anorexia, postprandial fullness, and chronic diarrhea with 2 to 3 daily bowel movements of nonformed stool. Her past medical history was unremarkable.
Physical examination. The patient’s vital signs were within normal limits. Physical examination revealed an ill-appearing, pale, cachectic woman.
Diagnostic tests. Results of laboratory tests, including a complete blood cell count, a basic metabolic panel, urinalysis, and thyrotropin, were normal with the exception of hypoproteinemia (5.4 g/dL), hypoalbuminemia (2.0 g/dL), and anemia (hemoglobin of 8.5 g/dL). Results of iron studies were consistent with anemia of chronic disease. Chest radiography revealed clear lung fields, and electrocardiography showed normal sinus rhythm with low-voltage QRS complexes.
Computed tomography (CT) of the abdomen and pelvis revealed hepatomegaly and a 2.1 × 1.8-cm liver mass that was solid-appearing, homogenous, and well-circumscribed. Triple-phase chest CT revealed bilateral pleural effusions, a 2.4-cm left breast mass, and an “indeterminate” liver mass. Colonoscopy revealed a 1.5-cm pedunculated rectal mass. All colon biopsy findings were reported as normal.
Esophagogastroduodenoscopy (EGD) revealed a large amount of undigested food, consistent with gastroparesis, despite her nothing-by-mouth (NPO) status for more than 24 hours. She was discharged from the hospital in stable condition.
Progression. Within 2 months, the patient was nonambulatory. Mammography and breast biopsy results were negative for cancer. She was readmitted to the hospital where another EGD revealed undigested food despite her being NPO for 48 hours. Biopsy results of the liver mass were reported as hemangioma with sinusoidal lymphocytosis. Results of additional tests for autoimmune and endocrine etiologies, including tests for Scl-70 antibodies, antinuclear antibodies, antitissue transglutaminase antibodies, 24-hour urine 5-hydroxyindoleacetic acid, adrenocorticotropic hormone, and cortisol, were unrevealing.
Within 2 weeks, the patient’s renal function had declined. Amyloidosis was suggested because of the paradoxical symptoms of gastroparesis and a highly motile lower gastrointestinal (GI) tract. Multiple myeloma as a unifying diagnosis was considered in light of the strong suspicion for malignancy. Serum protein electrophoresis and urine protein electrophoresis results showed a paraprotein spike in the gamma region; subsequent immunofixation revealed an immunoglobulin G λ monoclonal protein. Bone marrow biopsy showed neoplastic cells accounting for 80% of the overall marrow cellularity (Figures 1 and 2). Focal amyloid deposition in the bone marrow was confirmed by Congo red staining. Features were suggestive of cyclin D1 translocation. Flow cytometry results were significant for 18.1% plasma cells, immunotypes of which were CD20+, CD38+, and CD56+.
Multiple myeloma and immunoglobulin light chain (AL) amyloidosis was diagnosed. Reexamination of the patient’s colonic biopsy revealed amyloid deposition with apple-green birefringence on Congo red staining (Figures 3 and 4). The patient’s liver biopsy was also reexamined; what originally had been thought to be a hemangioma with lymphocytosis was determined to be a plasmacytoma with λ light-chain deposition (Figures 5 and 6).
Figures: Pathologic tissue ndings in multiple myeloma with AL amyloidosis. Bone marrow biopsy with hematoxylin-eosin staining (1) and CD138 staining (2) demonstrated a plasma cell occupation of greater than 80%. Colon biopsy with Congo red staining (3) showed amyloid deposition and apple-green birefringence under polarized light (4). Liver mass biopsy with CD138 staining (5) and immunohistochemistry staining for λ light-chain (6) demonstrated circulating plasma cells consistent with plasmacytoma.
Outcome of the case. The patient was immediately started on chemotherapy but died 1 week later.
Discussion. Multiple myeloma classically presents with signs and symptoms related to plasma cell infiltration of bone, lytic bone lesions, or renal damage from protein deposition.
In our patient’s unusual case of multiple myeloma, she presented with a protein-losing gastroenteropathy manifesting as profound weight loss, chronic diarrhea, and fatigue. Superimposed AL amyloidosis resulted in amyloid protein deposition in the GI tract, leading to severe gastroparesis and malabsorption.
Many of the classic signs of multiple myeloma were not initially evident in this patient, making for an elusive diagnosis. This case demonstrates the multisystem involvement that can occur in multiple myeloma with AL amyloidosis, with GI tract, liver, and bone marrow involvement ultimately discovered.
Multiple myeloma should be considered in the differential diagnosis for patients with protein-losing gastroenteropathy and chronic GI dysmotility.
Sean Sims, PA-C
Waynesboro Family Medical Associates, Waynesboro, Pennsylvania
Andrew Lininger, CRNP
Summit Gastroenterology, Waynesboro, Pennsylvania
A 56-year-old woman presented to her primary care provider for evaluation of a lump in her left axilla, which she had noticed while showering. She described the lump as painful, enlarging, and having been present for 1 month. She reported that pain radiated down her left arm, and she also noted ongoing fatigue, which had kept her in bed.
Her history was positive for smoking approximately 1 pack of cigarettes a day for 40 years, as well as for thyroid cancer with subsequent partial thyroidectomies but no other cancers.
Physical examination revealed fullness and an enlarged node in the patient’s left axilla. The results of a complete blood cell count were normal. She was referred to a general surgeon for a biopsy of the axillary node. She also was sent for thyroid function testing, the results of which returned as normal.
Less than 1 week later, she returned with concern for an odor to her urine and vaginal irritation. She also reported that her stool had become dark, which she attributed to having started taking an iron supplement. She had lost approximately 1.8 kg since her previous visit. Urinalysis results at this visit were normal. A lumbar spine series of radiographs was ordered, and she was given acetaminophen with codeine for pain relief.
The lumbar radiographs showed degenerative changes in the patient’s lower lumbar vertebrae, L5 pars defects, and diffuse reticular nodular infiltrates bilaterally in both lung bases (Figure 1). Chest radiography was ordered, the results of which revealed ubiquitous reticular densities in the lung fields bilaterally without effusion or hilar lymphadenopathy (Figure 2). Results of a computed tomography scan of her abdomen and pelvis showed a suspicious lesion in the tail of the pancreas (Figure 3).
Figure 1: Lumbar radiographs showed degenerative changes in the patient’s lower lumbar vertebrae, L5 pars defects, and numerous reticular densities in both lung bases (arrow).
Figure 2: Radiographs revealed ubiquitous reticular densities in the lung elds bilaterally without effusion or hilar lymphadenopathy.
Figure 3: Computed tomography scans of the abdomen and pelvis revealed a primary lesion in the tail of the pancreas (arrow).
Pathology test results of the axillary node biopsy specimen revealed stage IV metastatic cancer—specifically, a moderately differentiated adenocarcinoma of the pancreas. Results of an interferon-γ release assay for tuberculosis were negative, and results of a histoplasmosis blood test were negative for fungal infection with Histoplasma capsulatum. Her cancer antigen (CA) 19-9 level measured 6238 U/mL (reference value, < 37 U/mL).
The patient was referred to an oncologist to discuss treatment options; ultimately, she elected for a 2-drug combination palliative chemotherapy regimen of intravenous gemcitabine and paclitaxel. Surgical resection was not an option. She died approximately 6 months after the biopsy results confirmed the presence of metastatic pancreatic cancer.
Discussion. Pancreatic cancer is generally diagnosed late, is very painful, carries a poor prognosis, and is discovered as an incidental finding.1 For a small percentage of patients—approximately 5% to 25%—surgery is a treatment option.2 If resection is not an option, a patient can elect to either undergo chemotherapy or have no treatment. Although surgical intervention was not indicated in our patient’s case of metastatic cancer, had the neoplasm been confined to the pancreas, it would have been an option that is associated with reduced morbidity and mortality rates in the first year.2
The mainstay of treatment for metastatic pancreatic cancer is combination chemotherapy aimed at improving overall survival. Survival rates typically peak at 20% for the first year compared with 3% to 7% at 5 years; the poor prognosis is related to the high likelihood of metastasis at time of diagnosis, making treatment with pancreatoduodenectomy not possible.3
CA 19-9 measurement is commonly used as an adjunct in the initial diagnostic workup of suspected cases of pancreatic cancer.4 An elevated presurgical CA 19-9 level has been found to be an independent predictor of high mortality after surgical resection in patients with biliary tract malignancies.5 Serial tumor marker tests were not performed in our patient’s case, but the results likely would have shown a progressive increase in the CA 19-9 level in the months leading to her diagnosis.6,7 However, the genotype of approximately 5% of the population makes them unable to produce CA 19-9, precluding its use as a biomarker for pancreatic cancer.4,5
- Wysocka O, Kulbacka J, Saczko J. Adjuvant, neoadjuvant, and experimental regimens in overcoming pancreatic ductal adenocarcinoma. Prz Gastroenterol. 2016;11(3):155-162.
- Baker EH, Ross SW, Seshadri R, et al. Robotic pancreaticoduodenectomy for pancreatic adenocarcinoma: role in 2014 and beyond. J Gastrointest Oncol. 2015;6(4):396-405.
- Jiao L, Li D. Epidemiology and prospects for prevention of pancreatic cancer. In: Neoptolemos JP, Urrutia R, Abbruzzese JL, Büchler MW, eds. Pancreatic Cancer. Vol 1. New York, NY: Springer; 2010:3-25.
- Goonetilleke KS, Siriwardena AK. Systematic review of carbohydrate antigen (CA 19-9) as a biochemical marker in the diagnosis of pancreatic cancer. Eur J Surg Oncol. 2007;33(3):266-270.
- Hatzaras I, Schmidt C, Muscarella P, Melvin WS, Ellison EC, Bloomston M. Elevated CA 19-9 portends poor prognosis in patients undergoing resection of biliary malignancies. HPB (Oxford). 2010;12(2):134-138.
- Tempero MA, Uchida E, Takasaki H, Burnett DA, Steplewski Z, Pour PM. Relationship of carbohydrate antigen 19-9 and Lewis antigens in pancreatic cancer. Cancer Res. 1987;47(20):5501-5503.
- Locker GY, Hamilton S, Harris J, et al. ASCO 2006 update of recommendations for the use of tumor markers in gastrointestinal cancer. J Clin Oncol. 2006;24(33):5313-5327.