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8/16/2014 » 8/17/2014
TSP YPS Retreat 2014

1/23/2015 » 1/25/2015
TSP 94th Annual Meeting

2012 Abstract Participants - Page 1
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2012 Abstract Participants: Page 1

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Number

Category

1-2 Bone & Soft Tissue
3-5 Cardiothoracic
6-8 Cytopathology
9-12 Dermatopathology
13-14 Gastrointestinal


Bone and Soft Tissue

1. Rare presentation of bone xanthoma in a child

Alyaa Al-Ibraheemi, MD
UT - Houston Medical School

Introduction: The term xanthoma classically refers to a lesion composed of lipid-laden histiocytes and is thought to occur as a reactive histiocyte proliferation in response to elevated lipid levels. Rare cases of xanthoma have been reported to occur in bone, usually in association with hyperlipidemia syndromes. We report a case of a 10-year old previously healthy Hispanic boy who presented with a pathologic fracture of the mid-shaft femur. Imaging revealed a well-defined lytic lesion at the fracture site.
Materials and Methods: Gross examination of the excisional biopsy and the curettage of the lesion revealed an aggregate of 8.0 x 5.0 x 1.7 cm of multiple brown and yellow soft tissue fragments admixed with blood clots and bone fragments, entirely submitted for histopathological evaluation.
Results: Microscopic examination showed abundant foamy macrophages intermixed with blood clots, benign fragments of bone and muscle. No spindle cell proliferation, cellular atypia, increased mitotic activity or malignancy was identified. Based on the microscopic findings along with the radiological images, a diagnosis of benign xanthoma of the femur bone was established.
Conclusion: Xanthoma of bone is a rare benign bone tumor, approximately less than 50 mostly adults cases reported in the axial and appendicular skeleton) that is often associated with familial hyperlipidemia disorders, although it may also occur as a primary lesion in the absence of dyslipidemia; consequently, the diagnosis should prompt a clinical work-up for disorders of lipid metabolism. Radiographically, xanthomas of bone are characterized by well-defined, lytic lesions with an expansile border that can often extend into surrounding soft tissues. Diagnostic pathologic features are identical to those of xanthomas found elsewhere and include abundant foamy macrophages and varying numbers of multinucleated giant cells. Xanthoma of bone should be distinguished from similar entities, such as benign fibrous histiocytoma, by a lack of spindle cell proliferation.

2. Extra-skeletal Osteosarcoma: A Case Report of a Rare Neoplasm

Megan Lockyer, DO
Baylor College of Medicine

Introduction: Extra-skeletal osteosarcoma is a rare mesenchymal, bone-forming lesion with a poor prognosis.  Patients have an estimated five year survival of 46% status post margin-free surgery.
Materials and Methods: We report a case of a 57 year old man with a painless, left posterior arm mass of eight years.
Results: Radiologic examination revealed a soft tissue mass in the posterior deltoid muscle with central stippled calcifications and areas of necrosis.  A diagnosis of atypical mesenchymal lesion with focal osseous matrix favoring extra-skeletal osteosarcoma was made from core biopsies.
Conclusion: The mainstay of treatment for this lesion is wide local excision due to its relative chemoresistance.  Since surgery can be curative in the absence of metastases, it is important to consider extra-skeletal osteosarcoma in the differential of a slow-growing, ossifying mass to allow for early diagnosis and complete excision before advanced-stage disease.


Cardiothoracic

3. A Rare Case of Metastatic Signet-Ring Cell Carcinoma of Lung Primary Mimicking Histiocytes on Immediate Cytopathologic Assessment

Kristine Konopka, MD
UT - Houston Medical School

Introduction: Signet-ring cell carcinoma (SRCC) is a subtype of mucin-producing adenocarcinoma with abundant accumulation of intracellular mucin, most frequently arising from stomach, colon or breast.  Case reports of primary SRCC of the lung are few, but have increased in recent years with reporting of immunohistochemical staining profiles.  Here, we demonstrate the utility of a focused approach to immunohistochemical workup when evaluating the origin of metastatic SRCC. The patient was a 58-year-old woman who presented complaining of a one-month history of sore throat, cough and hemoptysis, associated with a 10-pound weight loss.  Her past medical history was most significant for hypertension, diabetes mellitus type II and hyperlipidemia.  She never smoked.  Chest radiograph revealed  prominence of the aortic knob,  prompting evaluation by computed tomography angiogram (CTA).  CTA of the chest showed paratracheal, subcarinal and bilateral hilar lymphadenopathy (up to 2 cm), numerous patchy bilateral ground glass opacities, and a 1.3 cm left lower lobe subpleural nodule, concerning for neoplasm.  The patient proceeded to biopsy for further workup.
Materials and Methods: Ultrasound-guided core needle biopsy of an enlarged right supraclavicular lymph node was performed by Radiology. Two Diff-Quick and two Papanicolaou stains were prepared and reviewed by Cytopathology for immediate adequacy assessment.  The core needle biopsies were then placed in formalin for histological processing.
Results: Immediate cytopathologic assessment showed abundant cells with eccentrically placed nuclei and foamy cytoplasm, reminiscent of histiocytes. Subsequent hematoxylin and eosin sections of the core needle biopsy specimen demonstrated discohesive cells with nuclear pleomorphism and large mucin droplets that displaced the nucleus to the edge of the cell, morphologically consistent with metastatic poorly differentiated adenocarcinoma with signet-ring cell features.  Some malignant cells had a more granular-appearing cytoplasm. Immunohistochemical stains were positive for pan-cytokeratin (CK), CK7, thyroid transcription factor-1 and napsin A, and negative for CK20 and estrogen receptor. This staining pattern is most specific for a lung primary.
Conclusion: We report a rare case of SRCC of lung origin with challenging cytology, and present a targeted strategy for immunohistochemical workup of metastatic disease. The presence of signet-ring cells portends a worse prognosis than other lung adenocarcinomas, so a high index of suspicion is needed.

4. Angioedema of the Tongue and Recent Myocardial Infarction: Separate Entities or Natural Disease Process?

Vinita Magoon, DO
Texas A&M - Scott and White

Introduction: Angioedema is usually caused by allergies and is a well-known side effect of certain medications, such as angiotensin-converting enzyme (ACE)-inhibitors.  Mast cells, macrophages, eosinophils, and lymphocytes, have been implicated in the pathogenesis of angioedema.  Inflammatory mediators such as bradykinin, leukotrienes, and histamine are also major players in the process. Interestingly, myocardial infarctions also induce an inflammatory response that recruits inflammatory cells which release inflammatory mediators. Allergic reactions involving mast cell activation and inflammatory mediator release has been known to induce coronary artery vasospasm and progress to myocardial infarction, known as Kounis syndrome (or allergic myocardial infarction). However, can the opposite process occur, where a myocardial infarction with the activation of numerous cells and the release of inflammatory mediators causes an allergic-type reaction? Here we present a case of acute myocardial infarction presenting with angioedema of the tongue.
Materials and Methods: Medical records and history were reviewed.  An autopsy was performed including histologic studies.
Results: The decedent is a 58 year-old male who presented to the emergency department (ED) with angioedema of the tongue and soon after, suffered cardiopulmonary arrest.  At the ED, labs were as follows: troponin of 49 ng/ml, creatine kinase of 936 IU/L, and CK-MB of 36 ng/ml. White blood cell count was elevated at 21,000 x 103/?L with the differential showing increased neutrophils.  Eosinophils and basophils were not elevated.   At autopsy, there was hemopericardium due to a ruptured myocardial infarction. The coronary arteries had severe atherosclerosis with thrombotic occlusion of the right coronary artery. The tongue was grossly edematous with a myxoid appearance.  Microscopically, the myocytes demonstrated hypereosinophilia with loss of nuclei and dense interstitial neutrophilic infiltration, consistent with a recent myocardial infarction. The tongue demonstrated abundant neutrophils with occasional eosinophils in a background of edema, consistent with angioedema. Post-mortem tryptase level was 7.1 ?g/L.
Conclusion: Myocardial infarction elicits an inflammatory response.  Release of inflammatory mediators can create a variety of different symptoms.  In this case, angioedema of the tongue was most likely caused by the inflammatory response initiated by the myocardial infarction.

5. Sudden Cardiac Death in a Case of IgG4-Related Sclerosing Disease with Coronary Artery Involvement

Nimesh Patel, MD
Baylor College of Medicine

Introduction: IgG4-Related Sclerosing Disease (IgG4RSS) is a systemic disorder characterized by multi-organ fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy.  Previous reports have described the disease in the pancreas, salivary glands, orbit, kidney, and aorta, among other sites.  However, to our knowledge, involvement of the coronary arteries has not been previously reported.
Materials and Methods: A 53-year-old Hispanic male presented to the emergency center after he was found unresponsive on the floor of his apartment by his roommate.  Cardiopulmonary resuscitation was performed in the field and ACLS protocol was initiated in the hospital; however, the patient remained in asystole.  Autopsy was subsequently performed and the postmortem findings are presented.
Results: Autopsy revealed cardiomegaly (430 grams), left ventricular hypertrophy, and severe three-vessel coronary artery disease.  The proximal coronary arteries exhibited conspicuously thick walls and were encased by pink-tan, fleshy tissue, raising the concern for an infiltrative process.  Histologically, the proximal coronary arteries showed a combination of transmural lymphoplasmacytic infiltrate with fibrosis; atherosclerotic changes; and two-vessel thromboses.  This was associated with subacute myocardial infarction of the anterolateral left ventricular wall from the apex to the base.  Further examination revealed variable lymphoplasmacytic inflammation, eosinophilic infiltrate, and fibrosis of the abdominal lymph nodes, kidneys, pancreas, liver, and lungs.  Immunohistochemical staining for CD138 and IgG4 was performed on FFPE sections of the coronary arteries and representative kidney.  In both sites, there were greater than 50 IgG4-positive plasma cells per high power field in the most plasma-cell-rich areas.
Conclusion: The postmortem findings were consistent with multi-organ involvement by IgG4RSS.  This is the first case report of involvement of the coronary arteries by this disease.  The inflammation and sclerosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. In addition, two of the coronary arteries were further occluded by thrombosis.  These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death.  This case raises the concern for a severe complication of IgG4RSS, making coronary artery evaluation prudent in cases of suspected IgG4RSS.


Cytopathology

6. Renal Epithelioid Angiomyolipoma with marked atypia and nuclear pseudoinclusions: A potential pitfall on touch prep and frozen section

Komal Arora, MD
UT - San Antonio HSC

Introduction: Epithelioid angiomyolipoma is a variant of angiomyolipoma characterized by predominance of epithelioid cells with abundant granular cytoplasm, pleomorphic nuclei, and prominent nucleoli. The cytologic atypia may be pronounced leading to mistaken diagnosis of a sarcomatoid renal cell carcinoma (RCC), melanoma or sarcoma.
Materials and Methods: We describe the touch preparation and corresponding frozen section findings of a renal epithelioid angiomyolipoma.
Results: The patient is a 54-year-old male who presented with hematuria and a renal mass.  A CT scan showed 11 x 6.7 x 6.6 cm heterogeneous, well-circumscribed mass with peripheral fat attenuation at the superior pole of the kidney with extension into the renal pelvis and associated cortical thinning. The patient underwent laparoscopic nephrectomy with a request for frozen section.  The nephrectomy specimen showed a 13.0 x 7.0 x 6.0 cm circumscribed, focally hemorrhagic, tan-yellow tumor located at the superior pole.  Cytologic preparations were imprinted from the tumor.  The H & E stained smears revealed numerous singly scattered to loosely cohesive large atypical cells.  The individual cells had single to multinucleated, round to oval nuclei with intranuclear inclusions, very prominent nucleoli and moderate to abundant granular cytoplasm. Multinucleated giant cells including Touton and Langhan s type were present.  The corresponding frozen histologic section tissue showed presence of markedly atypical epithelioid cells with hyperchromatic nuclei, prominent nucleoli and moderate eosinophilic  to bubbly cytoplasm focally intimately associated with lipoblast-like cells.  Multinucleated giant cells and nuclear pseudoinclusions were also present. Based on the touch preparations and histologic findings on the frozen sections, a diagnosis of malignant neoplasm was rendered on frozen section. The permanent histologic sections showed markedly atypical cells morphologically similar to those seen in the touch preparations.  Immunohistochemically, these atypical cells were positive for HMB-45 and negative for S-100, AE1/AE3 and SMA.  A final diagnosis of epithelioid angiomyolipoma with atypia was rendered.
Conclusion: The cytologic features of epithelioid angiomyolipoma should be recognized as it can mimic morphologically a variety of neoplasms including sarcomatoid RCC, adrenal cortical neoplasm, sarcoma and melanoma.  Due to their potential malignant behavior, close follow-up is recommended.

7. Pilomatrixoma mimicking a squamous cell carcinoma on fine needle aspiration:  A case report

Laura Been, MD
Baylor College of Medicine

Introduction: Pilomatrixoma is a benign adnexal tumor which typically presents as a subepidermal nodule in the head, neck, and upper extremities of children and young adults. When a nodule occurs in the head and neck region of older adults, the differential diagnosis includes a malignant neoplasm, either primary or metastatic.
Materials and Methods: We report a case of a 64 year old male who presented with a three centimeter posterior neck mass which had been present for two years.  On physical exam the nodule was mobile, non-tender and no overlying skin changes were noted.  Imaging studies were suspicious for malignancy and the lesion was positive on positron emission tomography (PET) scan.  Fine needle aspiration biopsy with core needle biopsy was performed.
Results: The smears were cellular with cohesive sheets of basaloid cells with some evidence of keratinization.  Mild cytologic atypia with prominent nucleoli and occasional mitoses were noted. Core biopsy and cell block material showed similar findings and immunohistochemical stains were performed. The cells were positive for p63 and CK5/6 leading to a diagnosis of squamous cell carcinoma.  Re-evaluation of the pathology was requested by the treating physician who felt that this clinical presentation was not consistent with a diagnosis of squamous cell carcinoma.  Re-evaluation of the case demonstrated basaloid squamous cells in the presence of sheets of pale  ghost cells  and a rare giant cell. No necrosis was seen.  An amended diagnosis of pilomatrixoma was then rendered before any treatment was pursued.
Conclusion: Review of the literature revealed cautionary reports of the pitfalls in mistaking a benign pilomatrixoma as a malignant tumor on fine needle aspiration. Smears are often highly cellular consisting of small primitive cells with a high nuclear-cytoplasmic ratio, prominent nucleoli, nuclear molding and mitotic activity.  For these reasons, malignant neoplasms such as basal cell carcinoma, squamous cell carcinoma, Merkel cell carcinoma or other metastatic tumors are usually considered on the differential diagnosis. Caution is advised when rendering a malignant diagnosis on fine needle aspiration and one should always consider an atypical presentation of benign tumors such as a pilomatrixoma.

8. Malignant Paraganglioma: A case diagnosed by Fine Needle Aspiration

Vanessa Moreno, MD
UT - Houston Medical School

Introduction: The prevalence of malignant paragangliomas is approximately 10% and is defined by the presence of metastases. Differential diagnosis includes hepatic tumors, alveolar soft part sarcoma, melanoma, glomus tumors, vascular neoplasms, and primary or metastatic carcinomas with endocrine or nonendocrine phenotype. Increasingly, fine needle aspiration (FNA) is being used to evaluate both primary and potential metastatic lesions. We describe a recent case of metastatic paraganglioma diagnosed by FNA and review the relevant clinicopathologic features of this rare entity.
Materials and Methods: A 41 year-old male with past medical history of hypertension was admitted with left sided numbness and weakness for 3 days. A 5.7 cm mass was found on the right anterior chest with destruction of the anterior portion of the 6th rib. An FNA with core biopsies was obtained. CT abdomen/pelvis showed multiple nodules along the spinal ganglion chain representing primary paraganglioma. Pathologic material was obtained including 6 slides (3 Diff-Quick, and 3 Papanicolaou stains) and 6 fragmented red-tan needle cores. Immunostains for CD56, Synaptophysin, Chromogranin, Vimentin, CK7, CK20, AE1/AE3, CD10, CD34 and CAM5.2 were performed using standard techniques.
Results: Cytologic examination demonstrated irregular clusters of tumor cells in an organoid-like pattern with eosinophilic, finely granular cytoplasm and irregularly shaped nuclei with finely stippled, densely clumped chromatin. Vascular tracts were noted. Tumor cells were negative for keratin and positive for CD56, synaptophysin, chromogranin, and vimentin. S100 highlighted sustentacular cells. The microscopic and immunohistochemical findings along with the radiographic features were consistent with metastatic malignant paraganglioma.
Conclusion: Malignant paraganglioma is a rare entity with a 40-72% 5-year survival. Patients with increased (>5cm) primary tumor size or with primary tumors located in the mediastinum or infradiaphragmatic paraaortic area are at increased risk for malignancy. Our case demonstrates that these patients can be reliably diagnosed using a combination of cytology, immunohistochemistry, clinical history, and imaging.


Dermatopathology

9. Cancer of an Unknown Primary Presenting as an Axillary Mass in a 55 Year old Male

Maria Gonzalez, MD
UT - Houston Medical School

Introduction: We present a case of a 55 year old male who presented with thrombosis of the right axillary vein.  He was found to have an axillary mass.  Additional scans found hypodense lesions in the pancreas and thyroid.   CEA, CA 19-9, PSA, AFP, BHCG were all within normal limits.  An FNA was performed  on the right axilla and was interpreted as positive for malignancy.   Tumor cells were strongly positive for CK7 and moderately positive for PSA.  They were negative for  CK20,  synaptophysin, chromogranin, and TTF-1.   Tissue from the right axillary LN was sent to  Cancer type ID  to further classify the tissue of origin.  The test was  inconclusive with a 62% probability of salivary gland origin.  The patient was treated with Carboplatin/Taxol with a presumptive diagnosis of salivary gland carcinoma.  Despite chemotherapy, the patient s lesions continued to increase in size and the right axillary mass was removed one year later.
Materials and Methods: The right axillary mass with attached skin ellipse was grossed and sections of tumor were taken for histologic evaluation.  Immunohistochemical (IHC) stains were performed for CK 20, CK 7, CK 5/6, CEA, TTF-1, S-100, CDX2, Estrogen receptor, GCDFP, SMA, and vimentin. Dr. Dehner from Washington University was consulted.
Results: The axillary dissection (8.5 x 8.5 x 5.5 cm) with attached skin ellipse contained  an 8.5 x 5.5x 5.0 cm mass.  The dermis contained  pleomorphic cells arranged in nests, cords and sheets with frequent mitotic figures and areas of necrosis. Lymphovascular invasion was present.  IHC stains were positive for CK-7, GCDFP and negative for CK 20, CK 5/6, CEA, TTF-1, S-100, CDX2, ER, SMA and vimentin. The case was originally signed out as poorly differentiated carcinoma with a differential diagnosis of apocrine sweat gland carcinoma and less likely salivary gland carcinoma or breast carcinoma.  The expert consultant signed the case as sweat gland (apocrine) carcinoma Grade III/III.
Conclusion: This patient has a complex history with an axillary mass of unknown origin. The tumor was poorly-differentiated with a differential diagnosis that was broad.  Appropriate clinical correlation, and thorough workup including histology and immunohistochemistry should supersede conclusions based on molecular tests.

10. Metastatic Melanoma with Multiple Patterns Mimicking Intradermal Nevus and Traumatic Neuroma: A Possible Diagnostic Pitfall

Justin Hardin, MD
Methodist Hospital Program

Introduction: Nevoid melanomas represent a rare and diagnostically challenging variety of malignant melanoma.  These lesions can be easily mistaken for benign nevi on first impression at low-power examination.  Although no single finding is considered pathognomonic for nevoid melanoma, careful attention to cytologic details (expansile pattern of growth and mitotic figures in the deep dermis) should raise the index of suspicion.  Despite their typical, relatively bland histologic appearance, these lesions seem to present the same prognosis and risk of metastases as conventional melanoma.
Materials and Methods: We report a case of a 68 year-old woman with a remote history of melanoma of the calf.  The patient now presented with multiple pigmented lesions on the lower leg clinically suspicious for regional metastases.
Results: A biopsy of one of these lesions showed an area of melanocytes in the dermis suggestive of intradermal nevus. A separate punch showed an area with clusters of slender spindle cells with minimal pleomorphism and nerves in the deep dermis and subcutaneous tissue, mimicking a neurofibroma.  A separate nodule was composed of spindle and epithelioid cells with large nuclei and prominent nucleoli.  In neither one of the three areas was there melanin pigment. The melanocytic nature of the three areas was confirmed by detection of melanocytic markers (HMB45 antigen and MART1) by immunohistochemistry, thus consistent with metastatic melanoma.
Conclusion: This case reports illustrates that metastatic melanoma can take on a wide array of histologic appearances, sometimes mimicking benign entities. To our knowledge, this is the first report concerning such disparate morphologies in late-onset metastatic melanoma.

11. Not your typical skin nodule: Clear cell adenocarcinoma arising in scar endometriosis

Andrea Haws, MD
Baylor College of Medicine

Introduction: A 47 year-old-woman presented with an eight month history of a tender subcutaneous nodule over the left end of a C-section scar, which gradually grew with time, and became associated with daily menstrual-like cramping pain (6/10).
Materials and Methods: Six months prior to presentation, the subcutaneous nodule was thought to be a cyst, at which time it was incised and drained, but it quickly returned and continued growing.  Upon presentation, examination of the left paramedian abdomen showed a 2 cm friable, red, vascular, ulcerated nodule, with palpable larger subcutaneous extension.  The clinical differential diagnosis included cutaneous metastasis, pyogenic granuloma, cutaneous endometriosis, and amelanotic malignant melanoma.
Results: A punch biopsy was performed, which showed a clear cell adenocarcinoma.  The tumor cells were immunoreactive with pancytokeratin, vimentin, estrogen receptor, CA-125 and MOC-31, and no reactivity with S-100, WT1, RCC, CD10 and p63, supporting an endometrial origin.  Upon resection, the tumor showed similar morphology to the punch biopsy; however, a single focus of endometriosis with focal atypia contiguous with clear cell adenocarcinoma was identified.  Imaging and examination did not find tumor in the ovaries or the uterus.
Conclusion: Malignant transformation of endometriosis associated with an abdominal wall scar is rare, with only a handful of reports in the literature. We report a case of 47 year old woman with a subcutaneous abdominal wall mass diagnosed as clear cell adenocarcinoma arising from endometriosis within a C-section scar, followed by a review of the literature for this unusual entity.

12. High grade adenocarcinoma presenting as a vulvar metastasis

Amanda Tchakarov, MD
UT - Houston Medical School

Introduction: The incidence of cutaneous metastases is low at 5.3%; the incidence of cutaneous metastasis as a presenting symptom is even lower at 0.8%. When a high grade adenocarcinoma with an unknown primary presents as a cutaneous metastasis, it can present a diagnostic challenge to the Pathologist. We present a case of a 56 year-old woman who presented with exquisitely tender nodules in her groin along with edematous labia majora. Skin biopsy of the area resulted in a diagnosis of high grade adenocarcinoma with unknown primary.
Materials and Methods: 4 mm suprapubic punch biopsy which was analyzed by hematoxylin and eosin as well as immunostains for CK 5/6, CK 7, CK AE1/AE3, CK20, CD 56, CEA, CDX-2, E-Cadherin, ER, PR, P16, p53, Ki-67, S100, HMB45, GCDFP, TTF1 and HPV.
Results: The biopsy showed unremarkable epidermis with diffuse dermal infiltration by a high-grade adenocarcinoma with desmoplastic stromal reaction. The immunophenotype was positive for CK 7, CK AE1/AE3, CK20, CEA, E-Cadherin, P16, p53 and focally CDX2, along with a high Ki-67 index. It was negative for S100, HMB45, GCDFP, ER, PR, CD56, CK 5/6, TTF-1 and HPV.  This led to a final diagnosis of diffuse dermal infiltration by high-grade adenocarcinoma suggestive of skin adnexal tumor origin without the ability to rule out metastatic tumor.
Conclusion: After pathological diagnosis was made, the patient was radiographically studied which revealed likely metastatic pulmonary nodules along with possible vertebral metastasis. On being informed of her possible metastatic cancer, the patient revealed that she had been treated for colorectal adenocarcinoma approximately 2 years prior. On review of the case, this poorly differentiated adenocarcinoma is compatible with metastasis from a colorectal primary. This case illustrates an unusual situation of previously treated adenocarcinoma presenting with cutaneous metastasis and demonstrates the diagnostic difficulty faced when the primary tumor is of unknown origin.


Gastrointestinal

13. Fatal Clostridium difficile Enteritis in a Patient with Familial Adenomatous Polyposis

Andrew Chu, MD
Baylor University Medical Center

Introduction: Clostridium difficile is an anaerobic, Gram-positive, spore-forming bacillus. It is a widely recognized colonic pathogen, most commonly associated with pseudomembranous colitis. In fact, 0.5% of hospitalized patients develop Clostridium difficile colitis, making it the leading cause of nosocomial infections. However, Clostridium difficile enteritis (small bowel) is very uncommon. We report the first case of Clostridium difficile enteritis in a patient with familial adenomatous polyposis (FAP).
Materials and Methods: A 42-year-old man with a history of familial adenomatous polyposis underwent total proctocolectomy and end ileostomy 20 years ago for colon carcinoma. Thereafter, he underwent numerous adhesiolysis procedures.  The patient recently developed abdominal pain around his ileostomy site. Subsequently, he underwent parastomal hernia repair and ileostomy revision and received surgical prophylaxis with ertapenem.
Results: Despite an uneventful surgery and initially unremarkable postoperative course, the patient was hospitalized three days status post surgery for abdominal pain, nausea, and vomiting; he went on to develop fatal Clostridium difficile enteritis.
Conclusion: Clostridium difficile enteritis is very uncommon. It has been hypothesized that the peristalsis of the small bowel in conjunction with the mechanical action of the ileocecal valve prevents colonic flora from colonizing the small bowel. Accordingly, in almost all cases of Clostridium difficile enteritis, the patient has had a history of surgical resection of the colon. Additionally, concomitant antibiotic use disturbs the normal bowel flora predisposing the patient to Clostridium difficile overgrowth. Physicians must be aware of the possibility of Clostridium difficile enteritis even when evaluating patients who have undergone prior colectomy. The signs and symptoms most characteristic of Clostridium difficile infection are diarrhea, abdominal pain, previous antibiotic use, and fever. It is important to recognize that in patients with ostomies, diarrhea will manifest as increased ostomy output. The rarity of Clostridium difficile enteritis leads to delays in diagnosis and subsequently to increased mortality rates (60%) when compared with Clostridium difficile colitis (2%).

14. Hepatobiliary Cystadenoma in a woman with a previously aspirated liver cyst and benign ovarian dermoid cyst: A Case Report

Elizabeth Jacobi
UT - Houston Medical School

Introduction: Hepatobiliary cystadenoma is a rare, benign, cystic lesion of the liver. It accounts for 1% of all hepatic cystic lesions and 5% of all symptomatic cystic lesions. Interestingly, it is significantly more common in women (85-95%). Much of what is known of this entity is derived from single case reports and case series in the literature. Our case is one of a 34 year old Caucasian woman with a significant past medical history of a previously aspirated hepatic cyst (pathologic diagnosis unknown), Hepatitis B (HBV) infection, and a benign dermoid cyst of the left ovary, which was removed. To our knowledge, there have been no reported cases of hepatobiliary cystadenoma with concurrence of both HBV and benign dermoid tumor. This patient had a 6 month history of progressive abdominal pain, weight gain and increased girth, early satiety and decreased appetite, bilateral lower extremity edema, and increasing shortness of breath while supine. She had CT imaging done at an outside hospital and upon viewing the images, a large, septated cystic structure occupying the entire left lobe of the liver was seen. The patient was then sent to our facility for higher level of care. Based on the CT imaging, the patient was taken to the operating room where subsequent frozen section identified the cystic structure as a hepatobiliary cystadenoma. Left hemihepatectomy was then performed to remove the cyst. Sections were then taken for formal histological analysis and immunohistochemical staining. The results are presented in this report.
Materials and Methods: N/A
Results: This is a case of a 34 year old, obese, Caucasian woman with a past medical history of hepatic cyst, HBV positivity, and benign dermoid cyst, who first presented to an outside hospital with a six month history of abdominal pain, weight gain, and increasing abdominal girth. The patient s past medical history was significant for a ruptured hepatic cyst, HBV infection, hypercholesterolemia in 1998, and a benign mature dermoid cyst of the left ovary in 2001. Surgical history included CT guided percutaneous aspiration of the hepatic cyst in 1998, left salpingo-oopherectomy in for removal of benign dermoid cyst in 2001, two cesarean sections (2001 and 2004), and a right tubal ligation in 2004. The patient is adopted but her family history was unremarkable. Patient stated that she was not on any medications at the time of admission. Abdominal CT with contrast was performed at the outside hospital. The CT scan revealed a large cystic structure occupying the entire left lobe of the liver.  The lesion was hypoechoic, multiloculated, septated, and had a thickened, irregular wall. The patient was then transferred to our facility for higher level of care. The patient then went to the operating room where a left hemihepatectomy was performed for complete removal of the cyst. Histological sections showed a single layer of epithelium with cuboidal to columnar, mucus secreting cells with a surrounding dense, fibrous stoma with spindle like cells in a whorled pattern that resembled ovarian stroma. Surrounding this ovarian-like stroma was another layer of dense, more linear, collagenous type stroma that separated the ovarian-like stroma from the adjacent normal liver parenchyma. There were also areas of the epithelial lining that showed invagination with budding of the lining, giving rise to numerous daughter cysts. We then performed multiple immunohistochemical (IHC) stains including CK7, CK19, ER, PR, Inhibin, p53, and Ki67. CK7 and CK19 were strongly positive in the epithelial lining of the cyst. ER was positive in the underlying stroma of the cyst and weakly positive in focal areas of liver parenchyma. PR staining was positive in the cyst stroma. Inhibin, which is a hormone that is secreted by the granulosa cells of the ovary and Sertoli cells of the testes, was strongly positive in the spindle like stroma directly beneath the epithelial lining of the cyst. The p53 staining has patchy positivity in both the underlying stroma and epithelium of the cyst. There was also patchy positivity of p53 in the liver parenchyma which is to be expected since the liver is a regenerative organ. Ki67, a marker of proliferation, showed patchy positivity in the epithelial lining of the cyst.
Conclusion: Hepatobiliary cystadenoma is a rare hepatic cystic lesion that occurs most often in middle age women. Little has been reported about this entity and etiology is still unknown. However, there are case reports, including this one that may provide clues to the origin of this hepatic lesion. The evidence supports that there is some sex specific factor involved in the pathophysiology. These cysts occur most often in women (85-95%) and the hepatobiliary cystadenoma with mesenchymal stroma occur exclusively  in women. Our patient s history is unique in the fact that she has had a previous ovarian lesion and a recurrent hepatic cystic lesion, both of which are hormonally responsive. IHC staining performed (ER, PR, Inhibin positivity) on sections of the hepatobiliary cystadenoma provides more support to this hypothesis. The CK19 and CK7 suggest that this cyst is originating from the biliary epithelium. Taking both these pieces of data into account, there may be some hormonal factor that precipitates the formation of these cysts in the liver. The fact that our patient has a history of benign ovarian dermoid cyst may also suggest that the same underlying pathophysiology may be involved in the formation of both entities, or perhaps the hepatobiliary cystadenoma may be derived from cystic properties of the ovaries. Although underlying etiology is still unknown, it is important to determine the origin of this entity, which may provide physicians with information on how to prevent or treat, with less invasive measures, these types of lesions in the future.


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